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of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;6&#44;9&#44;10</span></a> It is caused by recombination between two <span class="elsevierStyleItalic">MER67B</span> repeats in introns 3 and 10 of the gene&#44; resulting in loss of protein function&#46; Small insertion-deletion and nonsense mutations have also been described&#46; Some hypomorphic <span class="elsevierStyleItalic">IKBKG</span>&#47;<span class="elsevierStyleItalic">NEMO</span> mutations cause a distinct entity&#58; hypohidrotic ectodermal dysplasia with immunodeficiency &#40;OMIM 300291&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">6&#44;11</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Epidemiology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Incontinentia pigmenti has an estimated incidence of 0&#46;7 cases per 100&#160;000 births<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> and 27&#46;6 new cases annually&#46; Between 65&#37; and 75&#37; of cases are due to sporadic mutations and the remaining cases are familial&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">2</span></a> The condition is usually lethal in hemizygous male embryos&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;4&#44;12&#44;13</span></a> Female patients can survive due to functional mosaicism resulting from X chromosome inactivation&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#44;4&#44;9</span></a> The clinical findings in such cases are highly variable&#46;<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> Incontinentia pigmenti is therefore predominant in females&#44; with a female to male ratio of 37&#58;1&#46; Male patients with clinical features of incontinentia pigmenti may also have Klinefelter syndrome &#40;47&#44; XXY&#41;&#46; Survival in such cases is possible due to the second chromosome X or somatic mosaicism for the above-mentioned common deletion&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;5&#44;8</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Clinical Features and Management</span><p id="par0020" class="elsevierStylePara elsevierViewall">Incontinentia is a multisystemic disease<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;5&#44;8</span></a> that affects both ectodermal and mesodermal tissues&#46; The skin is always involved and is the main diagnostic criterion&#46;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">9</span></a> Additional alterations affect the central nervous system&#44; eyes&#44; teeth&#44;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;4&#44;10&#44;12</span></a> mammary glands&#44; hair&#44; nails&#44; and skeleton&#46; Cardiopulmonary alterations are also observed&#44; albeit less frequently&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">12</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Landy and Donnai<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">14</span></a> established the first diagnostic criteria for incontinentia pigmenti in 1993&#44; before discovery of the causative gene&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> In 2014&#44; Mini&#263; et al&#46;<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">15</span></a> proposed a series of updated criteria&#44; and in 2018&#44; with the permission of the authors&#44; Rosser<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> modified these criteria to improve the phenotypic characterization of the disease and further update the diagnostic criteria&#46; The major criteria proposed were the typical dermatologic findings observed in incontinentia pigmenti that are used to establish a clinical diagnosis&#44;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;5</span></a> while the minor criteria corresponded to other possible alterations&#46; Additional diagnostic criteria include a positive family history &#40;in a first-degree relative&#41; and a mutation in the <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> gene&#46; These are described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#44;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;20</span></a> together with the other criteria described by Rosser<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> and the respective frequencies as reported in the literature&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Skin lesions are the presenting manifestation&#46; They appear at birth or in the first 2 weeks of life&#44; progress over a period of years&#44; and are distributed along the Blaschko lines&#46; There are 4 classic stages&#58; a vesicobullous stage &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; a verrucous&#47;hyperkeratotic stage&#44; a hyperpigmented stage &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; and an atrophic&#47;hypopigmented stage&#46;<a class="elsevierStyleCrossRefs" href="#bib0215"><span class="elsevierStyleSup">19&#44;20</span></a> Not all patients experience the 4 stages&#44;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">19</span></a> and the first 3 can appear simultaneously&#46;<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">20</span></a> These lesions are described in more detail in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;20</span></a> The term <span class="elsevierStyleItalic">incontinentia pigmenti</span> is linked to the histologic characteristics of the lesions in stage 3 of the disease &#40;hyperpigmentation&#41; and specifically to melanin incontinence from melanocytes in the basal epidermal layer and deposits in the superficial dermis&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> These manifestations generally do not require specific intervention<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> or treatment&#46; Treatment with topical corticosteroids or tacrolimus can delay progression of the vesicobullous stage&#44; although the lesions resolve spontaneously&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Incontinence pigmenti largely becomes serious when there are neurological alterations&#44;<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">9&#44;10</span></a> as these can cause considerable morbidity and mortality&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">19</span></a> Neurologic impairments are varied in nature&#44; but the most common are seizures&#44;<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">9&#44;21</span></a> which are generally tonic<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">22</span></a> or focal clonic&#44; last for a short time&#44; and cause loss of consciousness&#46; They are more common in the neonatal period&#44;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">9</span></a> are resistant to treatment&#44; and have a poor prognosis&#46; Other disorders include microcephaly&#44; ischemic stroke&#44; cerebellar ataxia&#44; and delayed psychomotor development&#46; Acute signs of encephalitis mainly appear in the first 4 days of life and include food intolerance&#44; lethargy&#44; and seizure-like movements&#46; Brain magnetic resonance imaging with angiography is therefore essential in neonates with characteristic cutaneous incontinentia pigmenti lesions&#46; Follow-up studies are also necessary to prevent sequelae&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;9&#44;22</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Eye impairment is observed in between 50&#37; and 77&#37; of patients&#46; The most serious alterations involve the retina&#44;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">16</span></a> and retinal problems such as detachment and avascular retina are the most feared of complications&#46; These can be treated with laser photocoagulation targeting retinal neovascularization&#46; Cryotherapy can also be used and promising results have been observed with vascular endothelial growth factor inhibitors&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> Optical coherence tomography images show internal and external retinal layer thinning&#46; Eye abnormalities similar to retinoblastoma have been described in some cases of incontinentia pigmenti and include leukocoria&#44; strabismus&#44; intraocular calcification&#44; and&#44; as already mentioned&#44; retinal detachment&#46; The differential diagnosis is therefore important&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">16</span></a> Patients should have an eye examination every month for the first 4 months&#44; every 3 months up to 1 year of age&#44; every 6 months up to 3 years of age&#44; and every 12 months thereafter&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Etiology and Pathogenesis</span><p id="par0045" class="elsevierStylePara elsevierViewall">NF-&#954;B is activated by the protein product of <span class="elsevierStyleItalic">IKBKG&#47;</span><span class="elsevierStyleItalic">NEMO</span>&#44; which plays a key modulatory role in multiple physiological functions&#44; such as immune response and stress&#44; inflammatory reactions&#44; ectodermal development&#44; cell adhesion&#44; and protection of cells against tumor necrosis factor&#8211;induced apoptosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;4&#44;6&#44;22</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> mutation leads to decreased NF-&#954;B activity&#44; thereby increasing susceptibility to apoptosis&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> Extensive apoptosis in males is responsible for early fetal death<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> and liver disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Inflammatory reactions and epidermal recruitment of eosinophils&#44; which occur in the first stage of disease&#44; tend to have an important pathogenic role&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> This is probably related to eosinophil-selective chemokine &#40;eotaxin&#41;&#44;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;16</span></a> which is produced by specific leukocytes&#44; such as eosinophils&#44; macrophages&#44; and T cells&#44; and structural cells such as endothelial cells&#44; fibroblasts&#44; and epithelial cells&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Differential Diagnosis</span><p id="par0060" class="elsevierStylePara elsevierViewall">The entities that should be contemplated in the differential diagnosis vary with disease stage&#46; During the first stage&#44; incontinentia pigmenti may be confused with multiple infections of the newborn&#44; such as congenital herpes simplex&#44; varicella&#44; epidermolysis bullosa&#44; and bullous pemphigoid&#46;<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">18&#44;23</span></a> As neonatal herpes and incontinentia pigmenti can coexist&#44; treatment with acyclovir must be started as soon as a viral infection is confirmed&#46; The differential diagnosis in the second stage is limited and should include linear epidermal nevus&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">23</span></a> The third stage is the hallmark stage of incontinentia pigmenti and must be distinguished from linear and whorled nevoid hypermelanosis&#46; The fourth stage&#44; if present&#44; can be mistaken for hypomelanosis of Ito or vitiligo&#46; These conditions&#44; however&#44; lack an initial inflammatory phase and do not generally affect the appendages&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a></p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusions and Genetic Counseling</span><p id="par0065" class="elsevierStylePara elsevierViewall">The diagnosis of incontinentia pigmenti is based on clinical findings&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> Nonetheless&#44; in view of the enormous variability of clinical features and the broad spectrum of molecular alterations&#44; it is difficult to establish a homogeneous group of patients and standardize treatment&#46; Although progress has been made in our understanding of the causes of incontinentia pigmenti and in clinical care research&#44; the scarcity of patients seen in diagnostic centers makes it difficult to obtain a global epidemiological picture&#46; Integration of these data could make an important contribution to future scientific endeavours&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">When dealing with a multisystemic disorder&#44; long-term&#44; individualized&#44; multidisciplinary follow-up is necessary&#44; with evaluation by dermatologists&#44; neurologists&#44; ophthalmologists&#44; and dentists&#44; among others&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;24</span></a> Congenital hypothyroidism&#44; myasthenia gravis&#44; and Wilms tumor have been described in certain cases of incontinentia pigmenti&#44;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> and one group of authors who recently observed liver disease in a patient with incontinentia pigmenti suggested including hepatic evaluation in this setting&#44; even though they were unable to establish a link between the 2 conditions&#46;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">1</span></a> This could mean that even broader care may be warranted in patients with incontinentia pigmenti&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Moreover&#44; the updated diagnostic criteria proposed by Rosser<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> do not include skeletal alterations&#44; &#40;e&#46;g&#46;&#44; low stature&#44; hemivertebra&#44; kyphosis&#44; scoliosis&#44; supernumerary ribs&#44; hip dysplasia&#44; hemiatrophy&#44; club foot&#44; <a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> or toe syndactyly<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;10</span></a>&#41; or cardiopulmonary disorders &#40;e&#46;g&#46;&#44; interatrial communication&#44;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> left ventricular endomyocardial fibrosis&#44; tricuspid insufficiency&#44; tetralogy of Fallot&#44;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;17</span></a> or pulmonary hypertension&#44; even in the absence of cardiovascular alterations&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#44;4</span></a> Risk of recurrent infection is also not currently contemplated&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> Future updates will probably incorporate many of these conditions to cover the main clinical manifestations of this disease&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Genetic studies are essential for advancing our understanding of incontinentia pigmenti and confirming diagnoses through molecular diagnostic techniques&#44; particularly in doubtful cases&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> Prognosis is generally good&#44; but&#44; as already mentioned&#44; regular follow-up by a multidisciplinary team is important&#46; Genetic family counseling is also important in patients with an X-linked dominant disorder&#44;<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">18&#44;23</span></a> which is usually lethal in male patients&#46; Female patients in this case have a 50&#37; chance of having a daughter with incontinentia pigmenti&#46; Miscarriage occurs in approximately 50&#37; of male fetuses&#46; Males that do survive should be referred for a cytogenetic study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conflicts of Interest</span><p id="par0085" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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          "titulo" => "Introduction"
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          "titulo" => "Etiology and Pathogenesis"
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          "titulo" => "Differential Diagnosis"
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          "titulo" => "Conclusions and Genetic Counseling"
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            0 => "Incontinentia pigmenti"
            1 => "<span class="elsevierStyleItalic">IKBKG&#47;NEMO</span>"
            2 => "Clinical features"
            3 => "Diagnosis"
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            0 => "Incontinencia <span class="elsevierStyleItalic">pigmenti</span>"
            1 => "<span class="elsevierStyleItalic">IKBKG&#47;NEMO</span>"
            2 => "Cl&#237;nica"
            3 => "Diagn&#243;stico"
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        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Incontinentia pigmenti &#40;Bloch-Sulzberger syndrome&#41; is a rare neuroectodermal dysplasia&#46; It is an X-linked dominant disorder caused by mutations in the <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> gene on Xq28&#46; Approximately 80&#37; of patients have a deletion of exons 4 to 10&#46; Incontinentia pigmenti has an estimated incidence of 0&#46;7 cases per 100 000 births&#46; In hemizygous males&#44; it is usually lethal&#44; while in females&#44; it has a wide spectrum of clinical manifestations&#46; Incontinentia pigmenti is a multisystemic disease that invariably features skin changes&#46; These changes are the main diagnostic criteria and they evolve in 4 stages&#44; in association with other abnormalities affecting the central nervous system&#44; eyes&#44; teeth&#44; mammary glands&#44; hair&#44; nails&#44; skin&#44; and other parts of the body&#46; The aim of this brief review is to highlight the clinical features of this genodermatosis and underline the importance of case-by-case interdisciplinary management&#44; including genetic counseling&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La incontinencia <span class="elsevierStyleItalic">pigmenti</span> &#40;s&#237;ndrome de Bloch-Sulzberger&#41; es una displasia neuroectod&#233;rmica infrecuente&#44; con patr&#243;n de herencia ligado al X dominante&#44; causada por mutaciones en el gen <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> y se encuentra localizado en Xq28&#46; La deleci&#243;n del ex&#243;n 4 al 10 corresponde con la principal causa en aproximadamente el 80&#37; de los casos&#46; La incidencia estimada es de 0&#44;7 en 100&#46;000 nacimientos&#44; usualmente letal en hombres hemicigotos y en el sexo femenino puede exhibir hallazgos cl&#237;nicos variables&#46; Es una entidad multisist&#233;mica&#44; que incluye defectos en la piel&#44; siempre presente y principal criterio diagn&#243;stico&#44; la cual evoluciona en cuatro etapas&#44; asociada a alteraciones en el sistema nervioso central&#44; globo ocular&#44; dientes&#44; gl&#225;ndula mamaria&#44; pelo&#44; u&#241;as&#44; entre otros&#46; El objeto de esta breve revisi&#243;n es resaltar los hallazgos cl&#237;nicos de esta genodermatosis&#44; con la finalidad de brindar el seguimiento m&#233;dico individualizado e interdisciplinario que incluya un adecuado asesoramiento gen&#233;tico familiar&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Please cite this article as&#58; Cammarata-Scalisi F&#44; Fusco F&#44; Ursini MV&#46; Incontinencia <span class="elsevierStyleItalic">pigmenti</span>&#46; Actas Dermosifiliogr&#46; 2019&#59;110&#58;273&#8211;278&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Blister-like erythematous lesions on the extremities corresponding to stage I disease &#40;vesiculobullous lesions&#41;&#46;</p>"
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                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Criteria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Frequency&#44; &#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Major</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Typical skin lesions following the lines of Blaschko&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">100&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage I &#8212; vesiculobullous stage&#58; erythematous or inflammatory lesions characterized by vesicles&#44; bullae&#44; or pustules&#59; more common on extremities and scalp&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Present from birth to second week of life&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage II &#8212; verrucous&#47;hyperkeratotic stage&#58; hyperpigmented pustules or crusts&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Occurs from week 2 to week 6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage III &#8212; hyperpigmentation stage&#58; whorled hyperchromic macules&#59; predominant in intertriginous areas and on trunk&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">These appear between week 12 and 26 and can improve in adolescence or persist into adulthood&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage IV &#8212; atrophy&#47;hypopigmentation stage&#58; hypochromic macules and possibly alopecia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">This stage is not always present&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">The above stages may overlap and not necessarily follow the given order&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Minor</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Central nervous system&#47;neurologic impairments</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">30&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Seizures&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">40&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Spastic paralysis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Psychomotor delay&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">26&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mental retardation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">29&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microcephaly&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">11&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cerebral&#47;cerebellar atrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&#46;4<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microgyria&#47;polymicrogyria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypoplasia of the corpus callosum&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Basal ganglia alterations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Periventricular leukomalacia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hydrocephaly&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Porencephaly&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ischemic stroke&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Diffuse hemorrhagic necrosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Encephalomyelitis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Eye alterations</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Visual defects&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retinopathy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retinal detachment&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retinal vascular alteration&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Pigmentation disorders &#40;hyper&#47;hypopigmentation&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Optic atrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Foveal hypoplasia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retrolental fibroplasia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cataracts&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microphthalmia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">6&#46;4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Strabismus&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Nystagmus&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Dental alterations</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17-34&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Delayed primary tooth eruption&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">34&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Anodontia&#47;hypodontia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microdontia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Dental dystrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Abnormal &#40;conical&#41; shape&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">22&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Impacted teeth&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Diastema&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Malocclusion&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Palate disorders</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">High-arched palate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Mammary gland alterations</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Supernumerary nipple&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Hair abnormalities &#40;hair&#44; eyebrows&#44; eyelashes&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">28-38&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Alopecia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">9&#46;7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypertrichosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&#46;6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Nail disorders</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Dystrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">64&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Yellowing&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vertical or horizontal splitting&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Male miscarriage</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Typical histopathologic findings in skin</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Additional conditions for confirming diagnosis</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">No history of incontinentia pigmenti in a first-degree female relative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">If molecular diagnosis is not available&#44; at least 2 major criteria or 1 major criterion plus 1 minor criterion are needed to confirm diagnosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">An <span class="elsevierStyleItalic">IKBKG</span> mutation combined with any major or minor criterion confirms diagnosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">History of incontinentia pigmenti in a first-degree female relative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">One major criterion or 2 minor criteria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">In all cases&#44; diagnosis is supported by eosinophilia and skewed X-chromosome inactivation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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        "texto" => "<p id="par0090" class="elsevierStylePara elsevierViewall">We would like to thank the Incontinentia Pigmenti Genetic Biobank &#40;<a class="elsevierStyleInterRef" target="_blank" id="intr0010" href="http://www/">www</a>&#46; igb&#46; cnr&#46; it&#47; ipgb&#41;&#44; which forms part of the Biobanking and BioMolecular Resources Research Infrastructure-European Research Infrastructure Consortium &#40;BBRMI-ERIC&#41;&#44; and the CNR-DSB Flagship Project InterOmics&#46;We also thank Rosal&#237;a Gumina F&#44; Managing Director of the Library at the Instituto Aut&#243;nomo Hospital Universitario de Los Andes&#44; Universidad de Los Andes&#46;</p>"
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Practical Dermatology
Incontinentia Pigmenti
Incontinencia pigmenti
F. Cammarata-Scalisia,
Autor para correspondencia
francocammarata19@gmail.com

Corresponding author.
, F. Fuscob, M.V. Ursinib
a Unidad de Genética Médica, Departamento de Puericultura y Pediatría, Universidad de Los Andes, Mérida, Venezuela
b Institute of Genetics and Biophysics «Adriano Buzzati-Traverso», IGB-CNR, Nápoles, Italia
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Incontinentia pigmenti &#40;OMIM 308300&#41;&#44; a rare neuroectodermal dysplasia&#44;<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">1&#44;2</span></a> is an X-linked dominant disorder caused by mutations in the <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> gene &#40;GenBankNM&#95;003639&#46;3&#44; OMIM 300248&#41;&#44; formerly known as <span class="elsevierStyleItalic">NEMO</span>&#46; The gene is located on Xq28 and encodes a kappa light polypeptide gene enhancer in B cells&#44; kinase gamma&#44; which has a key role in the modulation of nuclear transcription factor kappa B &#40;NF-&#954;B&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">1&#44;3&#8211;7</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Incontinentia pigmenti was described by Bloch in 1926 and Sulzberger in 1928<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> and is hence also known as <span class="elsevierStyleItalic">Bloch&#8211;Sulzberger syndrome</span>&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">6&#44;8</span></a> The causative gene has high penetrance and variable expressivity&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> The 11&#46;7-kb deletion spanning exons 4 to 10 is the main cause of incontinentia pigmenti and is found in approximately 80&#37; of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;6&#44;9&#44;10</span></a> It is caused by recombination between two <span class="elsevierStyleItalic">MER67B</span> repeats in introns 3 and 10 of the gene&#44; resulting in loss of protein function&#46; Small insertion-deletion and nonsense mutations have also been described&#46; Some hypomorphic <span class="elsevierStyleItalic">IKBKG</span>&#47;<span class="elsevierStyleItalic">NEMO</span> mutations cause a distinct entity&#58; hypohidrotic ectodermal dysplasia with immunodeficiency &#40;OMIM 300291&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0150"><span class="elsevierStyleSup">6&#44;11</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Epidemiology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Incontinentia pigmenti has an estimated incidence of 0&#46;7 cases per 100&#160;000 births<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> and 27&#46;6 new cases annually&#46; Between 65&#37; and 75&#37; of cases are due to sporadic mutations and the remaining cases are familial&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">2</span></a> The condition is usually lethal in hemizygous male embryos&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;4&#44;12&#44;13</span></a> Female patients can survive due to functional mosaicism resulting from X chromosome inactivation&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#44;4&#44;9</span></a> The clinical findings in such cases are highly variable&#46;<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> Incontinentia pigmenti is therefore predominant in females&#44; with a female to male ratio of 37&#58;1&#46; Male patients with clinical features of incontinentia pigmenti may also have Klinefelter syndrome &#40;47&#44; XXY&#41;&#46; Survival in such cases is possible due to the second chromosome X or somatic mosaicism for the above-mentioned common deletion&#46;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;5&#44;8</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Clinical Features and Management</span><p id="par0020" class="elsevierStylePara elsevierViewall">Incontinentia is a multisystemic disease<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;5&#44;8</span></a> that affects both ectodermal and mesodermal tissues&#46; The skin is always involved and is the main diagnostic criterion&#46;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">9</span></a> Additional alterations affect the central nervous system&#44; eyes&#44; teeth&#44;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;4&#44;10&#44;12</span></a> mammary glands&#44; hair&#44; nails&#44; and skeleton&#46; Cardiopulmonary alterations are also observed&#44; albeit less frequently&#46;<a class="elsevierStyleCrossRef" href="#bib0180"><span class="elsevierStyleSup">12</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Landy and Donnai<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">14</span></a> established the first diagnostic criteria for incontinentia pigmenti in 1993&#44; before discovery of the causative gene&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> In 2014&#44; Mini&#263; et al&#46;<a class="elsevierStyleCrossRef" href="#bib0195"><span class="elsevierStyleSup">15</span></a> proposed a series of updated criteria&#44; and in 2018&#44; with the permission of the authors&#44; Rosser<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> modified these criteria to improve the phenotypic characterization of the disease and further update the diagnostic criteria&#46; The major criteria proposed were the typical dermatologic findings observed in incontinentia pigmenti that are used to establish a clinical diagnosis&#44;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;5</span></a> while the minor criteria corresponded to other possible alterations&#46; Additional diagnostic criteria include a positive family history &#40;in a first-degree relative&#41; and a mutation in the <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> gene&#46; These are described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#44;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;20</span></a> together with the other criteria described by Rosser<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> and the respective frequencies as reported in the literature&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Skin lesions are the presenting manifestation&#46; They appear at birth or in the first 2 weeks of life&#44; progress over a period of years&#44; and are distributed along the Blaschko lines&#46; There are 4 classic stages&#58; a vesicobullous stage &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; a verrucous&#47;hyperkeratotic stage&#44; a hyperpigmented stage &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; and an atrophic&#47;hypopigmented stage&#46;<a class="elsevierStyleCrossRefs" href="#bib0215"><span class="elsevierStyleSup">19&#44;20</span></a> Not all patients experience the 4 stages&#44;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">19</span></a> and the first 3 can appear simultaneously&#46;<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">20</span></a> These lesions are described in more detail in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;20</span></a> The term <span class="elsevierStyleItalic">incontinentia pigmenti</span> is linked to the histologic characteristics of the lesions in stage 3 of the disease &#40;hyperpigmentation&#41; and specifically to melanin incontinence from melanocytes in the basal epidermal layer and deposits in the superficial dermis&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> These manifestations generally do not require specific intervention<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> or treatment&#46; Treatment with topical corticosteroids or tacrolimus can delay progression of the vesicobullous stage&#44; although the lesions resolve spontaneously&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Incontinence pigmenti largely becomes serious when there are neurological alterations&#44;<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">9&#44;10</span></a> as these can cause considerable morbidity and mortality&#46;<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">19</span></a> Neurologic impairments are varied in nature&#44; but the most common are seizures&#44;<a class="elsevierStyleCrossRefs" href="#bib0165"><span class="elsevierStyleSup">9&#44;21</span></a> which are generally tonic<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">22</span></a> or focal clonic&#44; last for a short time&#44; and cause loss of consciousness&#46; They are more common in the neonatal period&#44;<a class="elsevierStyleCrossRef" href="#bib0165"><span class="elsevierStyleSup">9</span></a> are resistant to treatment&#44; and have a poor prognosis&#46; Other disorders include microcephaly&#44; ischemic stroke&#44; cerebellar ataxia&#44; and delayed psychomotor development&#46; Acute signs of encephalitis mainly appear in the first 4 days of life and include food intolerance&#44; lethargy&#44; and seizure-like movements&#46; Brain magnetic resonance imaging with angiography is therefore essential in neonates with characteristic cutaneous incontinentia pigmenti lesions&#46; Follow-up studies are also necessary to prevent sequelae&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;9&#44;22</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Eye impairment is observed in between 50&#37; and 77&#37; of patients&#46; The most serious alterations involve the retina&#44;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">16</span></a> and retinal problems such as detachment and avascular retina are the most feared of complications&#46; These can be treated with laser photocoagulation targeting retinal neovascularization&#46; Cryotherapy can also be used and promising results have been observed with vascular endothelial growth factor inhibitors&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> Optical coherence tomography images show internal and external retinal layer thinning&#46; Eye abnormalities similar to retinoblastoma have been described in some cases of incontinentia pigmenti and include leukocoria&#44; strabismus&#44; intraocular calcification&#44; and&#44; as already mentioned&#44; retinal detachment&#46; The differential diagnosis is therefore important&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">16</span></a> Patients should have an eye examination every month for the first 4 months&#44; every 3 months up to 1 year of age&#44; every 6 months up to 3 years of age&#44; and every 12 months thereafter&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Etiology and Pathogenesis</span><p id="par0045" class="elsevierStylePara elsevierViewall">NF-&#954;B is activated by the protein product of <span class="elsevierStyleItalic">IKBKG&#47;</span><span class="elsevierStyleItalic">NEMO</span>&#44; which plays a key modulatory role in multiple physiological functions&#44; such as immune response and stress&#44; inflammatory reactions&#44; ectodermal development&#44; cell adhesion&#44; and protection of cells against tumor necrosis factor&#8211;induced apoptosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;4&#44;6&#44;22</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> mutation leads to decreased NF-&#954;B activity&#44; thereby increasing susceptibility to apoptosis&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> Extensive apoptosis in males is responsible for early fetal death<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> and liver disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Inflammatory reactions and epidermal recruitment of eosinophils&#44; which occur in the first stage of disease&#44; tend to have an important pathogenic role&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> This is probably related to eosinophil-selective chemokine &#40;eotaxin&#41;&#44;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;16</span></a> which is produced by specific leukocytes&#44; such as eosinophils&#44; macrophages&#44; and T cells&#44; and structural cells such as endothelial cells&#44; fibroblasts&#44; and epithelial cells&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Differential Diagnosis</span><p id="par0060" class="elsevierStylePara elsevierViewall">The entities that should be contemplated in the differential diagnosis vary with disease stage&#46; During the first stage&#44; incontinentia pigmenti may be confused with multiple infections of the newborn&#44; such as congenital herpes simplex&#44; varicella&#44; epidermolysis bullosa&#44; and bullous pemphigoid&#46;<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">18&#44;23</span></a> As neonatal herpes and incontinentia pigmenti can coexist&#44; treatment with acyclovir must be started as soon as a viral infection is confirmed&#46; The differential diagnosis in the second stage is limited and should include linear epidermal nevus&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">23</span></a> The third stage is the hallmark stage of incontinentia pigmenti and must be distinguished from linear and whorled nevoid hypermelanosis&#46; The fourth stage&#44; if present&#44; can be mistaken for hypomelanosis of Ito or vitiligo&#46; These conditions&#44; however&#44; lack an initial inflammatory phase and do not generally affect the appendages&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a></p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusions and Genetic Counseling</span><p id="par0065" class="elsevierStylePara elsevierViewall">The diagnosis of incontinentia pigmenti is based on clinical findings&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> Nonetheless&#44; in view of the enormous variability of clinical features and the broad spectrum of molecular alterations&#44; it is difficult to establish a homogeneous group of patients and standardize treatment&#46; Although progress has been made in our understanding of the causes of incontinentia pigmenti and in clinical care research&#44; the scarcity of patients seen in diagnostic centers makes it difficult to obtain a global epidemiological picture&#46; Integration of these data could make an important contribution to future scientific endeavours&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">When dealing with a multisystemic disorder&#44; long-term&#44; individualized&#44; multidisciplinary follow-up is necessary&#44; with evaluation by dermatologists&#44; neurologists&#44; ophthalmologists&#44; and dentists&#44; among others&#46;<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">3&#44;24</span></a> Congenital hypothyroidism&#44; myasthenia gravis&#44; and Wilms tumor have been described in certain cases of incontinentia pigmenti&#44;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> and one group of authors who recently observed liver disease in a patient with incontinentia pigmenti suggested including hepatic evaluation in this setting&#44; even though they were unable to establish a link between the 2 conditions&#46;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">1</span></a> This could mean that even broader care may be warranted in patients with incontinentia pigmenti&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">Moreover&#44; the updated diagnostic criteria proposed by Rosser<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">3</span></a> do not include skeletal alterations&#44; &#40;e&#46;g&#46;&#44; low stature&#44; hemivertebra&#44; kyphosis&#44; scoliosis&#44; supernumerary ribs&#44; hip dysplasia&#44; hemiatrophy&#44; club foot&#44; <a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> or toe syndactyly<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;10</span></a>&#41; or cardiopulmonary disorders &#40;e&#46;g&#46;&#44; interatrial communication&#44;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">4</span></a> left ventricular endomyocardial fibrosis&#44; tricuspid insufficiency&#44; tetralogy of Fallot&#44;<a class="elsevierStyleCrossRefs" href="#bib0140"><span class="elsevierStyleSup">4&#44;17</span></a> or pulmonary hypertension&#44; even in the absence of cardiovascular alterations&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#44;4</span></a> Risk of recurrent infection is also not currently contemplated&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">10</span></a> Future updates will probably incorporate many of these conditions to cover the main clinical manifestations of this disease&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Genetic studies are essential for advancing our understanding of incontinentia pigmenti and confirming diagnoses through molecular diagnostic techniques&#44; particularly in doubtful cases&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">18</span></a> Prognosis is generally good&#44; but&#44; as already mentioned&#44; regular follow-up by a multidisciplinary team is important&#46; Genetic family counseling is also important in patients with an X-linked dominant disorder&#44;<a class="elsevierStyleCrossRefs" href="#bib0210"><span class="elsevierStyleSup">18&#44;23</span></a> which is usually lethal in male patients&#46; Female patients in this case have a 50&#37; chance of having a daughter with incontinentia pigmenti&#46; Miscarriage occurs in approximately 50&#37; of male fetuses&#46; Males that do survive should be referred for a cytogenetic study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Conflicts of Interest</span><p id="par0085" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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          "identificador" => "xres1187719"
          "titulo" => "Abstract"
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        1 => array:2 [
          "identificador" => "xpalclavsec1107568"
          "titulo" => "Keywords"
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          "titulo" => "Resumen"
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        3 => array:2 [
          "identificador" => "xpalclavsec1107569"
          "titulo" => "Palabras clave"
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          "identificador" => "sec0005"
          "titulo" => "Introduction"
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        5 => array:2 [
          "identificador" => "sec0010"
          "titulo" => "Epidemiology"
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        6 => array:2 [
          "identificador" => "sec0015"
          "titulo" => "Clinical Features and Management"
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        7 => array:2 [
          "identificador" => "sec0020"
          "titulo" => "Etiology and Pathogenesis"
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        8 => array:2 [
          "identificador" => "sec0025"
          "titulo" => "Differential Diagnosis"
        ]
        9 => array:2 [
          "identificador" => "sec0030"
          "titulo" => "Conclusions and Genetic Counseling"
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        10 => array:2 [
          "identificador" => "sec0035"
          "titulo" => "Conflicts of Interest"
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        11 => array:2 [
          "identificador" => "xack405583"
          "titulo" => "Acknowledgments"
        ]
        12 => array:1 [
          "titulo" => "References"
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      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2018-05-04"
    "fechaAceptado" => "2018-10-13"
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          "clase" => "keyword"
          "titulo" => "Keywords"
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          "palabras" => array:5 [
            0 => "Incontinentia pigmenti"
            1 => "<span class="elsevierStyleItalic">IKBKG&#47;NEMO</span>"
            2 => "Clinical features"
            3 => "Diagnosis"
            4 => "Treatment"
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          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec1107569"
          "palabras" => array:5 [
            0 => "Incontinencia <span class="elsevierStyleItalic">pigmenti</span>"
            1 => "<span class="elsevierStyleItalic">IKBKG&#47;NEMO</span>"
            2 => "Cl&#237;nica"
            3 => "Diagn&#243;stico"
            4 => "Tratamiento"
          ]
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Incontinentia pigmenti &#40;Bloch-Sulzberger syndrome&#41; is a rare neuroectodermal dysplasia&#46; It is an X-linked dominant disorder caused by mutations in the <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> gene on Xq28&#46; Approximately 80&#37; of patients have a deletion of exons 4 to 10&#46; Incontinentia pigmenti has an estimated incidence of 0&#46;7 cases per 100 000 births&#46; In hemizygous males&#44; it is usually lethal&#44; while in females&#44; it has a wide spectrum of clinical manifestations&#46; Incontinentia pigmenti is a multisystemic disease that invariably features skin changes&#46; These changes are the main diagnostic criteria and they evolve in 4 stages&#44; in association with other abnormalities affecting the central nervous system&#44; eyes&#44; teeth&#44; mammary glands&#44; hair&#44; nails&#44; skin&#44; and other parts of the body&#46; The aim of this brief review is to highlight the clinical features of this genodermatosis and underline the importance of case-by-case interdisciplinary management&#44; including genetic counseling&#46;</p></span>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La incontinencia <span class="elsevierStyleItalic">pigmenti</span> &#40;s&#237;ndrome de Bloch-Sulzberger&#41; es una displasia neuroectod&#233;rmica infrecuente&#44; con patr&#243;n de herencia ligado al X dominante&#44; causada por mutaciones en el gen <span class="elsevierStyleItalic">IKBKG&#47;NEMO</span> y se encuentra localizado en Xq28&#46; La deleci&#243;n del ex&#243;n 4 al 10 corresponde con la principal causa en aproximadamente el 80&#37; de los casos&#46; La incidencia estimada es de 0&#44;7 en 100&#46;000 nacimientos&#44; usualmente letal en hombres hemicigotos y en el sexo femenino puede exhibir hallazgos cl&#237;nicos variables&#46; Es una entidad multisist&#233;mica&#44; que incluye defectos en la piel&#44; siempre presente y principal criterio diagn&#243;stico&#44; la cual evoluciona en cuatro etapas&#44; asociada a alteraciones en el sistema nervioso central&#44; globo ocular&#44; dientes&#44; gl&#225;ndula mamaria&#44; pelo&#44; u&#241;as&#44; entre otros&#46; El objeto de esta breve revisi&#243;n es resaltar los hallazgos cl&#237;nicos de esta genodermatosis&#44; con la finalidad de brindar el seguimiento m&#233;dico individualizado e interdisciplinario que incluya un adecuado asesoramiento gen&#233;tico familiar&#46;</p></span>"
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        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Please cite this article as&#58; Cammarata-Scalisi F&#44; Fusco F&#44; Ursini MV&#46; Incontinencia <span class="elsevierStyleItalic">pigmenti</span>&#46; Actas Dermosifiliogr&#46; 2019&#59;110&#58;273&#8211;278&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Blister-like erythematous lesions on the extremities corresponding to stage I disease &#40;vesiculobullous lesions&#41;&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Hyperchromic macules corresponding to stage III disease &#40;hyperpigmented lesions&#41;&#46;</p>"
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          "leyenda" => "<p id="spar9040" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleSup">a</span>Adapted from references <a class="elsevierStyleCrossRefs" href="#bib0130"><span class="elsevierStyleSup">2&#8211;20</span></a></p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Criteria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th><th class="td" title="table-head  " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Frequency&#44; &#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Major</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Typical skin lesions following the lines of Blaschko&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">100&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage I &#8212; vesiculobullous stage&#58; erythematous or inflammatory lesions characterized by vesicles&#44; bullae&#44; or pustules&#59; more common on extremities and scalp&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Present from birth to second week of life&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage II &#8212; verrucous&#47;hyperkeratotic stage&#58; hyperpigmented pustules or crusts&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Occurs from week 2 to week 6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage III &#8212; hyperpigmentation stage&#58; whorled hyperchromic macules&#59; predominant in intertriginous areas and on trunk&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">These appear between week 12 and 26 and can improve in adolescence or persist into adulthood&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stage IV &#8212; atrophy&#47;hypopigmentation stage&#58; hypochromic macules and possibly alopecia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">This stage is not always present&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">The above stages may overlap and not necessarily follow the given order&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleBold">Minor</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Central nervous system&#47;neurologic impairments</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">30&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Seizures&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">40&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Spastic paralysis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">16&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Psychomotor delay&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">26&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Mental retardation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">29&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microcephaly&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">11&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cerebral&#47;cerebellar atrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">13&#46;4<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microgyria&#47;polymicrogyria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypoplasia of the corpus callosum&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Basal ganglia alterations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Periventricular leukomalacia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hydrocephaly&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Porencephaly&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Ischemic stroke&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Diffuse hemorrhagic necrosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Encephalomyelitis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Eye alterations</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Visual defects&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retinopathy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">15&#46;9&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retinal detachment&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">8&#46;5&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retinal vascular alteration&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">5&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Pigmentation disorders &#40;hyper&#47;hypopigmentation&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Optic atrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Foveal hypoplasia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Retrolental fibroplasia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Cataracts&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microphthalmia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">6&#46;4&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Strabismus&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Nystagmus&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Dental alterations</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17-34&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Delayed primary tooth eruption&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">34&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Anodontia&#47;hypodontia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Microdontia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Dental dystrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Abnormal &#40;conical&#41; shape&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">22&#46;3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Impacted teeth&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">17&#46;2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Diastema&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Malocclusion&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Palate disorders</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">High-arched palate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Mammary gland alterations</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Supernumerary nipple&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Hair abnormalities &#40;hair&#44; eyebrows&#44; eyelashes&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">28-38&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Alopecia&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">9&#46;7&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Hypertrichosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">3&#46;6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Nail disorders</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Dystrophy&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="left" valign="top">64&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Yellowing&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Vertical or horizontal splitting&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Male miscarriage</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Typical histopathologic findings in skin</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Additional conditions for confirming diagnosis</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">No history of incontinentia pigmenti in a first-degree female relative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">If molecular diagnosis is not available&#44; at least 2 major criteria or 1 major criterion plus 1 minor criterion are needed to confirm diagnosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">An <span class="elsevierStyleItalic">IKBKG</span> mutation combined with any major or minor criterion confirms diagnosis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">History of incontinentia pigmenti in a first-degree female relative&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">One major criterion or 2 minor criteria&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">In all cases&#44; diagnosis is supported by eosinophilia and skewed X-chromosome inactivation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="table-entry  " align="" valign="top">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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              "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Brain atrophy&#46;</p>"
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        "descripcion" => array:1 [
          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Proposed Diagnostic Criteria for Incontinentia Pigmenti and Prevalence of Some of the Corresponding Conditions as Reported in the Literature&#46;<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a></p>"
        ]
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    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:24 [
            0 => array:3 [
              "identificador" => "bib0125"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:3 [
                  "comentario" => "1111&#47;ajd&#46;12805"
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "A novel IKBKG mutation in a patient with incontinentia pigmenti and features of hepatic ciliopathy"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "S&#46; Danescu"
                            1 => "C&#46; Has"
                            2 => "C&#46; Baican"
                            3 => "T&#46; M&#252;ller"
                            4 => "A&#46; Baican"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:3 [
                        "tituloSerie" => "Australas J Dermatol&#46;"
                        "fecha" => "2018"
                        "paginaInicial" => "10"
                      ]
                    ]
                  ]
                ]
              ]
            ]
            1 => array:3 [
              "identificador" => "bib0130"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Incontinentia pigmenti&#58; A summary review of this rare ectodermal dysplasia with neurologic manifestations&#44; including treatment protocols"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "C&#46; Greene-Roethke"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1016/j.pedhc.2017.07.003"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Pediatr Health Care&#46;"
                        "fecha" => "2017"
                        "volumen" => "31"
                        "paginaInicial" => "e45"
                        "paginaFinal" => "e52"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28870493"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0135"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Neurocutaneous disorders"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "T&#46; Rosser"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1212/CON.0000000000000562"
                      "Revista" => array:6 [
                        "tituloSerie" => "Continuum &#40;Minneap Minn&#41;&#46;"
                        "fecha" => "2018"
                        "volumen" => "24"
                        "paginaInicial" => "96"
                        "paginaFinal" => "129"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/29432239"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib0140"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Incontinentia pigmenti&#58; A case report of a complex systemic disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "S&#46; Gianfaldoni"
                            1 => "G&#46; Tchernev"
                            2 => "U&#46; Wollina"
                            3 => "T&#46; Lotti"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.3889/oamjms.2017.128"
                      "Revista" => array:6 [
                        "tituloSerie" => "Open Access Maced J Med Sci&#46;"
                        "fecha" => "2017"
                        "volumen" => "5"
                        "paginaInicial" => "501"
                        "paginaFinal" => "505"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28785344"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
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            4 => array:3 [
              "identificador" => "bib0145"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Unusual father-to-daughter transmission of incontinentia pigmenti due to mosaicism in IP males"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "F&#46; Fusco"
                            1 => "M&#46;I&#46; Conte"
                            2 => "A&#46; Diociaiuti"
                            3 => "S&#46; Bigoni"
                            4 => "M&#46;F&#46; Branda"
                            5 => "A&#46; Ferlini"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1542/peds.2016-2950"
                      "Revista" => array:5 [
                        "tituloSerie" => "Pediatrics&#46;"
                        "fecha" => "2017"
                        "volumen" => "140"
                        "paginaInicial" => "e20162950"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28794079"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib0150"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Cognitive-behavioural phenotype in a group of girls from 1&#46;2 to 12 years old with the incontinentia pigmenti syndrome&#58; Recommendations for clinical management"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "M&#46;R&#46; Pizzamiglio"
                            1 => "L&#46; Piccardi"
                            2 => "F&#46; Bianchini"
                            3 => "L&#46; Canzano"
                            4 => "L&#46; Palermo"
                            5 => "F&#46; Fusco"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1080/21622965.2016.1188388"
                      "Revista" => array:6 [
                        "tituloSerie" => "Appl Neuropsychol Child&#46;"
                        "fecha" => "2017"
                        "volumen" => "6"
                        "paginaInicial" => "327"
                        "paginaFinal" => "334"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27267212"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
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            6 => array:3 [
              "identificador" => "bib0155"
              "etiqueta" => "7"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti&#46; The International Incontinentia Pigmenti &#40;IP&#41; Consortium"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46; Smahi"
                            1 => "G&#46; Courtois"
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        "texto" => "<p id="par0090" class="elsevierStylePara elsevierViewall">We would like to thank the Incontinentia Pigmenti Genetic Biobank &#40;<a class="elsevierStyleInterRef" target="_blank" id="intr0010" href="http://www/">www</a>&#46; igb&#46; cnr&#46; it&#47; ipgb&#41;&#44; which forms part of the Biobanking and BioMolecular Resources Research Infrastructure-European Research Infrastructure Consortium &#40;BBRMI-ERIC&#41;&#44; and the CNR-DSB Flagship Project InterOmics&#46;We also thank Rosal&#237;a Gumina F&#44; Managing Director of the Library at the Instituto Aut&#243;nomo Hospital Universitario de Los Andes&#44; Universidad de Los Andes&#46;</p>"
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