Incontinentia Pigmenti | Actas Dermo-Sifiliográficas

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Una revisión para dermatólogos" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1462 "Ancho" => 2500 "Tamanyo" => 252455 ] ] "descripcion" => array:1 [ "en" => "Vitamin D Metabolism. The body acquires vitamin D from certain foods and through a reaction that occurs when the skin is exposed to sunlight in which UV-B radiation converts 7-dehydrocholersterol (synthesized in the skin) to pre-vitamin D3, which then becomes inactive vitamin D. This is then converted in the liver to 25(OH)D by way of hepatic hydroxylation. In a second step, 25(OH)D is metabolized in the kidney to the active metabolite 1,25(OH)D, or calcitriol, through the action of renal alpha-1-hydroxylase. This mechanism is regulated by serum levels of calcium and inorganic phosphorus, among others, which are detected by the glands that secrete parathyroid hormone (PTH) and stimulate the activity of alpha-1-hydroxylase, when necessary. Calcitriol concentrations regulate the secretion of PTH. The 1,25(OH)D acts through the vitamin D receptors (VDR) within cells, which activate the transcription and translation of messenger RNA, leading to the synthesis of vitamin D-dependent proteins and their corresponding activity, which depend on the function of the target cell. The excretion of vitamin D through bile is mediated by the activity of 24 renal hydroxylase, which converts 1,25(OH)D to calcitroic acid." ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "F.J. Navarro-Triviño, S. Arias-Santiago, Y. 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Cammarata-Scalisi, F. Fusco, M.V. Ursini" "autores" => array:3 [ 0 => array:4 [ "nombre" => "F." "apellidos" => "Cammarata-Scalisi" "email" => array:1 [ 0 => "francocammarata19@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "F." "apellidos" => "Fusco" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "M.V." "apellidos" => "Ursini" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "b" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Unidad de Genética Médica, Departamento de Puericultura y Pediatría, Universidad de Los Andes, Mérida, Venezuela" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Institute of Genetics and Biophysics «Adriano Buzzati-Traverso», IGB-CNR, Nápoles, Italia" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Incontinencia pigmenti" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 803 "Ancho" => 1667 "Tamanyo" => 90787 ] ] "descripcion" => array:1 [ "en" => "Hyperchromic macules corresponding to stage III disease (hyperpigmented lesions)." ] ] ] "textoCompleto" => "IntroductionIncontinentia pigmenti (OMIM 308300), a rare neuroectodermal dysplasia,<a class="elsevierStyleCrossRefs" href="#bib0125">1,2</a> is an X-linked dominant disorder caused by mutations in the IKBKG/NEMO gene (GenBankNM_003639.3, OMIM 300248), formerly known as NEMO. The gene is located on Xq28 and encodes a kappa light polypeptide gene enhancer in B cells, kinase gamma, which has a key role in the modulation of nuclear transcription factor kappa B (NF-κB).<a class="elsevierStyleCrossRefs" href="#bib0125">1,3–7</a>Incontinentia pigmenti was described by Bloch in 1926 and Sulzberger in 1928<a class="elsevierStyleCrossRef" href="#bib0140">4</a> and is hence also known as Bloch–Sulzberger syndrome.<a class="elsevierStyleCrossRefs" href="#bib0150">6,8</a> The causative gene has high penetrance and variable expressivity.<a class="elsevierStyleCrossRef" href="#bib0140">4</a> The 11.7-kb deletion spanning exons 4 to 10 is the main cause of incontinentia pigmenti and is found in approximately 80% of cases.<a class="elsevierStyleCrossRefs" href="#bib0135">3,6,9,10</a> It is caused by recombination between two MER67B repeats in introns 3 and 10 of the gene, resulting in loss of protein function. Small insertion-deletion and nonsense mutations have also been described. Some hypomorphic IKBKG/NEMO mutations cause a distinct entity: hypohidrotic ectodermal dysplasia with immunodeficiency (OMIM 300291).<a class="elsevierStyleCrossRefs" href="#bib0150">6,11</a>EpidemiologyIncontinentia pigmenti has an estimated incidence of 0.7 cases per 100 000 births<a class="elsevierStyleCrossRef" href="#bib0170">10</a> and 27.6 new cases annually. Between 65% and 75% of cases are due to sporadic mutations and the remaining cases are familial.<a class="elsevierStyleCrossRef" href="#bib0130">2</a> The condition is usually lethal in hemizygous male embryos.<a class="elsevierStyleCrossRefs" href="#bib0130">2–4,12,13</a> Female patients can survive due to functional mosaicism resulting from X chromosome inactivation.<a class="elsevierStyleCrossRefs" href="#bib0130">2,4,9</a> The clinical findings in such cases are highly variable.<a class="elsevierStyleCrossRef" href="#bib0135">3</a> Incontinentia pigmenti is therefore predominant in females, with a female to male ratio of 37:1. Male patients with clinical features of incontinentia pigmenti may also have Klinefelter syndrome (47, XXY). Survival in such cases is possible due to the second chromosome X or somatic mosaicism for the above-mentioned common deletion.<a class="elsevierStyleCrossRefs" href="#bib0140">4,5,8</a>Clinical Features and ManagementIncontinentia is a multisystemic disease<a class="elsevierStyleCrossRefs" href="#bib0140">4,5,8</a> that affects both ectodermal and mesodermal tissues. The skin is always involved and is the main diagnostic criterion.<a class="elsevierStyleCrossRef" href="#bib0165">9</a> Additional alterations affect the central nervous system, eyes, teeth,<a class="elsevierStyleCrossRefs" href="#bib0135">3,4,10,12</a> mammary glands, hair, nails, and skeleton. Cardiopulmonary alterations are also observed, albeit less frequently.<a class="elsevierStyleCrossRef" href="#bib0180">12</a>Landy and Donnai<a class="elsevierStyleCrossRef" href="#bib0190">14</a> established the first diagnostic criteria for incontinentia pigmenti in 1993, before discovery of the causative gene.<a class="elsevierStyleCrossRef" href="#bib0170">10</a> In 2014, Minić et al.<a class="elsevierStyleCrossRef" href="#bib0195">15</a> proposed a series of updated criteria, and in 2018, with the permission of the authors, Rosser<a class="elsevierStyleCrossRef" href="#bib0135">3</a> modified these criteria to improve the phenotypic characterization of the disease and further update the diagnostic criteria. The major criteria proposed were the typical dermatologic findings observed in incontinentia pigmenti that are used to establish a clinical diagnosis,<a class="elsevierStyleCrossRefs" href="#bib0135">3,5</a> while the minor criteria corresponded to other possible alterations. Additional diagnostic criteria include a positive family history (in a first-degree relative) and a mutation in the IKBKG/NEMO gene. These are described in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>,<a class="elsevierStyleCrossRefs" href="#bib0130">2–20</a> together with the other criteria described by Rosser<a class="elsevierStyleCrossRef" href="#bib0135">3</a> and the respective frequencies as reported in the literature.<elsevierMultimedia ident="tbl0005"></elsevierMultimedia>Skin lesions are the presenting manifestation. They appear at birth or in the first 2 weeks of life, progress over a period of years, and are distributed along the Blaschko lines. There are 4 classic stages: a vesicobullous stage (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), a verrucous/hyperkeratotic stage, a hyperpigmented stage (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), and an atrophic/hypopigmented stage.<a class="elsevierStyleCrossRefs" href="#bib0215">19,20</a> Not all patients experience the 4 stages,<a class="elsevierStyleCrossRef" href="#bib0215">19</a> and the first 3 can appear simultaneously.<a class="elsevierStyleCrossRef" href="#bib0220">20</a> These lesions are described in more detail in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>.<a class="elsevierStyleCrossRefs" href="#bib0130">2–20</a> The term incontinentia pigmenti is linked to the histologic characteristics of the lesions in stage 3 of the disease (hyperpigmentation) and specifically to melanin incontinence from melanocytes in the basal epidermal layer and deposits in the superficial dermis.<a class="elsevierStyleCrossRef" href="#bib0140">4</a> These manifestations generally do not require specific intervention<a class="elsevierStyleCrossRef" href="#bib0135">3</a> or treatment. Treatment with topical corticosteroids or tacrolimus can delay progression of the vesicobullous stage, although the lesions resolve spontaneously.<a class="elsevierStyleCrossRef" href="#bib0210">18</a><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia>Incontinence pigmenti largely becomes serious when there are neurological alterations,<a class="elsevierStyleCrossRefs" href="#bib0165">9,10</a> as these can cause considerable morbidity and mortality.<a class="elsevierStyleCrossRef" href="#bib0215">19</a> Neurologic impairments are varied in nature, but the most common are seizures,<a class="elsevierStyleCrossRefs" href="#bib0165">9,21</a> which are generally tonic<a class="elsevierStyleCrossRef" href="#bib0230">22</a> or focal clonic, last for a short time, and cause loss of consciousness. They are more common in the neonatal period,<a class="elsevierStyleCrossRef" href="#bib0165">9</a> are resistant to treatment, and have a poor prognosis. Other disorders include microcephaly, ischemic stroke, cerebellar ataxia, and delayed psychomotor development. Acute signs of encephalitis mainly appear in the first 4 days of life and include food intolerance, lethargy, and seizure-like movements. Brain magnetic resonance imaging with angiography is therefore essential in neonates with characteristic cutaneous incontinentia pigmenti lesions. Follow-up studies are also necessary to prevent sequelae.<a class="elsevierStyleCrossRefs" href="#bib0135">3,9,22</a>Eye impairment is observed in between 50% and 77% of patients. The most serious alterations involve the retina,<a class="elsevierStyleCrossRef" href="#bib0200">16</a> and retinal problems such as detachment and avascular retina are the most feared of complications. These can be treated with laser photocoagulation targeting retinal neovascularization. Cryotherapy can also be used and promising results have been observed with vascular endothelial growth factor inhibitors.<a class="elsevierStyleCrossRef" href="#bib0210">18</a> Optical coherence tomography images show internal and external retinal layer thinning. Eye abnormalities similar to retinoblastoma have been described in some cases of incontinentia pigmenti and include leukocoria, strabismus, intraocular calcification, and, as already mentioned, retinal detachment. The differential diagnosis is therefore important.<a class="elsevierStyleCrossRef" href="#bib0200">16</a> Patients should have an eye examination every month for the first 4 months, every 3 months up to 1 year of age, every 6 months up to 3 years of age, and every 12 months thereafter.<a class="elsevierStyleCrossRef" href="#bib0210">18</a>Etiology and PathogenesisNF-κB is activated by the protein product of IKBKG/NEMO, which plays a key modulatory role in multiple physiological functions, such as immune response and stress, inflammatory reactions, ectodermal development, cell adhesion, and protection of cells against tumor necrosis factor–induced apoptosis.<a class="elsevierStyleCrossRefs" href="#bib0135">3,4,6,22</a>The IKBKG/NEMO mutation leads to decreased NF-κB activity, thereby increasing susceptibility to apoptosis.<a class="elsevierStyleCrossRef" href="#bib0140">4</a> Extensive apoptosis in males is responsible for early fetal death<a class="elsevierStyleCrossRef" href="#bib0170">10</a> and liver disorders.<a class="elsevierStyleCrossRef" href="#bib0130">2</a>Inflammatory reactions and epidermal recruitment of eosinophils, which occur in the first stage of disease, tend to have an important pathogenic role.<a class="elsevierStyleCrossRef" href="#bib0140">4</a> This is probably related to eosinophil-selective chemokine (eotaxin),<a class="elsevierStyleCrossRefs" href="#bib0140">4,16</a> which is produced by specific leukocytes, such as eosinophils, macrophages, and T cells, and structural cells such as endothelial cells, fibroblasts, and epithelial cells.<a class="elsevierStyleCrossRef" href="#bib0140">4</a>Differential DiagnosisThe entities that should be contemplated in the differential diagnosis vary with disease stage. During the first stage, incontinentia pigmenti may be confused with multiple infections of the newborn, such as congenital herpes simplex, varicella, epidermolysis bullosa, and bullous pemphigoid.<a class="elsevierStyleCrossRefs" href="#bib0210">18,23</a> As neonatal herpes and incontinentia pigmenti can coexist, treatment with acyclovir must be started as soon as a viral infection is confirmed. The differential diagnosis in the second stage is limited and should include linear epidermal nevus.<a class="elsevierStyleCrossRef" href="#bib0235">23</a> The third stage is the hallmark stage of incontinentia pigmenti and must be distinguished from linear and whorled nevoid hypermelanosis. The fourth stage, if present, can be mistaken for hypomelanosis of Ito or vitiligo. These conditions, however, lack an initial inflammatory phase and do not generally affect the appendages.<a class="elsevierStyleCrossRef" href="#bib0210">18</a>Conclusions and Genetic CounselingThe diagnosis of incontinentia pigmenti is based on clinical findings.<a class="elsevierStyleCrossRef" href="#bib0210">18</a> Nonetheless, in view of the enormous variability of clinical features and the broad spectrum of molecular alterations, it is difficult to establish a homogeneous group of patients and standardize treatment. Although progress has been made in our understanding of the causes of incontinentia pigmenti and in clinical care research, the scarcity of patients seen in diagnostic centers makes it difficult to obtain a global epidemiological picture. Integration of these data could make an important contribution to future scientific endeavours.<a class="elsevierStyleCrossRef" href="#bib0170">10</a>When dealing with a multisystemic disorder, long-term, individualized, multidisciplinary follow-up is necessary, with evaluation by dermatologists, neurologists, ophthalmologists, and dentists, among others.<a class="elsevierStyleCrossRefs" href="#bib0135">3,24</a> Congenital hypothyroidism, myasthenia gravis, and Wilms tumor have been described in certain cases of incontinentia pigmenti,<a class="elsevierStyleCrossRef" href="#bib0210">18</a> and one group of authors who recently observed liver disease in a patient with incontinentia pigmenti suggested including hepatic evaluation in this setting, even though they were unable to establish a link between the 2 conditions.<a class="elsevierStyleCrossRef" href="#bib0125">1</a> This could mean that even broader care may be warranted in patients with incontinentia pigmenti.Moreover, the updated diagnostic criteria proposed by Rosser<a class="elsevierStyleCrossRef" href="#bib0135">3</a> do not include skeletal alterations, (e.g., low stature, hemivertebra, kyphosis, scoliosis, supernumerary ribs, hip dysplasia, hemiatrophy, club foot, <a class="elsevierStyleCrossRef" href="#bib0140">4</a> or toe syndactyly<a class="elsevierStyleCrossRefs" href="#bib0140">4,10</a>) or cardiopulmonary disorders (e.g., interatrial communication,<a class="elsevierStyleCrossRef" href="#bib0140">4</a> left ventricular endomyocardial fibrosis, tricuspid insufficiency, tetralogy of Fallot,<a class="elsevierStyleCrossRefs" href="#bib0140">4,17</a> or pulmonary hypertension, even in the absence of cardiovascular alterations).<a class="elsevierStyleCrossRefs" href="#bib0130">2,4</a> Risk of recurrent infection is also not currently contemplated.<a class="elsevierStyleCrossRef" href="#bib0170">10</a> Future updates will probably incorporate many of these conditions to cover the main clinical manifestations of this disease.Genetic studies are essential for advancing our understanding of incontinentia pigmenti and confirming diagnoses through molecular diagnostic techniques, particularly in doubtful cases.<a class="elsevierStyleCrossRef" href="#bib0210">18</a> Prognosis is generally good, but, as already mentioned, regular follow-up by a multidisciplinary team is important. Genetic family counseling is also important in patients with an X-linked dominant disorder,<a class="elsevierStyleCrossRefs" href="#bib0210">18,23</a> which is usually lethal in male patients. Female patients in this case have a 50% chance of having a daughter with incontinentia pigmenti. Miscarriage occurs in approximately 50% of male fetuses. Males that do survive should be referred for a cytogenetic study.Conflicts of InterestThe authors declare that they have no conflicts of interest." "textoCompletoSecciones" => array:1 [ "secciones" => array:13 [ 0 => array:3 [ "identificador" => "xres1187719" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1107568" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1187720" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1107569" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Epidemiology" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Clinical Features and Management" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Etiology and Pathogenesis" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Differential Diagnosis" ] 9 => array:2 [ "identificador" => "sec0030" "titulo" => "Conclusions and Genetic Counseling" ] 10 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of Interest" ] 11 => array:2 [ "identificador" => "xack405583" "titulo" => "Acknowledgments" ] 12 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-05-04" "fechaAceptado" => "2018-10-13" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1107568" "palabras" => array:5 [ 0 => "Incontinentia pigmenti" 1 => "IKBKG/NEMO" 2 => "Clinical features" 3 => "Diagnosis" 4 => "Treatment" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1107569" "palabras" => array:5 [ 0 => "Incontinencia pigmenti" 1 => "IKBKG/NEMO" 2 => "Clínica" 3 => "Diagnóstico" 4 => "Tratamiento" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a rare neuroectodermal dysplasia. It is an X-linked dominant disorder caused by mutations in the IKBKG/NEMO gene on Xq28. Approximately 80% of patients have a deletion of exons 4 to 10. Incontinentia pigmenti has an estimated incidence of 0.7 cases per 100 000 births. In hemizygous males, it is usually lethal, while in females, it has a wide spectrum of clinical manifestations. Incontinentia pigmenti is a multisystemic disease that invariably features skin changes. These changes are the main diagnostic criteria and they evolve in 4 stages, in association with other abnormalities affecting the central nervous system, eyes, teeth, mammary glands, hair, nails, skin, and other parts of the body. 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, including genetic counseling." ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "La incontinencia pigmenti (síndrome de Bloch-Sulzberger) es una displasia neuroectodérmica infrecuente, con patrón de herencia ligado al X dominante, causada por mutaciones en el gen IKBKG/NEMO y se encuentra localizado en Xq28. La deleción del exón 4 al 10 corresponde con la principal causa en aproximadamente el 80% de los casos. La incidencia estimada es de 0,7 en 100.000 nacimientos, usualmente letal en hombres hemicigotos y en el sexo femenino puede exhibir hallazgos clínicos variables. Es una entidad multisistémica, que incluye defectos en la piel, siempre presente y principal criterio diagnóstico, la cual evoluciona en cuatro etapas, asociada a alteraciones en el sistema nervioso central, globo ocular, dientes, glándula mamaria, pelo, uñas, entre otros. El objeto de esta breve revisión es resaltar los hallazgos clínicos de esta genodermatosis, con la finalidad de brindar el seguimiento médico individualizado e interdisciplinario que incluya un adecuado asesoramiento genético familiar." ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "Please cite this article as: Cammarata-Scalisi F, Fusco F, Ursini MV. Incontinencia pigmenti. Actas Dermosifiliogr. 2019;110:273–278." ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1158 "Ancho" => 1667 "Tamanyo" => 168889 ] ] "descripcion" => array:1 [ "en" => "Blister-like erythematous lesions on the extremities corresponding to stage I disease (vesiculobullous lesions)." ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 803 "Ancho" => 1667 "Tamanyo" => 90787 ] ] "descripcion" => array:1 [ "en" => "Hyperchromic macules corresponding to stage III disease (hyperpigmented lesions)." ] ] 2 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "aAdapted from references <a class="elsevierStyleCrossRefs" href="#bib0130">2–20</a>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <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 \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, % \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">Major \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">100 \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 — vesiculobullous stage: erythematous or inflammatory lesions characterized by vesicles, bullae, or pustules; more common on extremities and scalp \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 — verrucous/hyperkeratotic stage: hyperpigmented pustules or crusts \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 — hyperpigmentation stage: whorled hyperchromic macules; predominant in intertriginous areas and on trunk \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 — atrophy/hypopigmentation stage: hypochromic macules and possibly alopecia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Minor \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Central nervous system/neurologic impairments \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">30 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">40.2 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">16.5 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">26 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">29.9 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11.3 \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/cerebellar atrophy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">13.4<a class="elsevierStyleCrossRef" href="#tblfn0005">a</a> \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/polymicrogyria \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.1 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Eye alterations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">17 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">15.9 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8.5 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.3 \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 (hyper/hypopigmentation) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6.4 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 alterations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">17-34 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">34.3 \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/hypodontia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">17.2 \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 (conical) shape \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">22.3 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">17.2 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Palate disorders \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Mammary gland alterations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Hair abnormalities (hair, eyebrows, eyelashes) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">28-38 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">9.7 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3.6 \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">Nail disorders \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">64 \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Male miscarriage \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 histopathologic findings in skin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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">Additional conditions for confirming diagnosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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, at least 2 major criteria or 1 major criterion plus 1 minor criterion are needed to confirm diagnosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 IKBKG mutation combined with any major or minor criterion confirms diagnosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \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, diagnosis is supported by eosinophilia and skewed X-chromosome inactivation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="" valign="top"> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2026139.png" ] ] ] "notaPie" => array:1 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "Brain atrophy." ] ] ] "descripcion" => array:1 [ "en" => "Proposed Diagnostic Criteria for Incontinentia Pigmenti and Prevalence of Some of the Corresponding Conditions as Reported in the Literature.<a class="elsevierStyleCrossRef" href="#tblfn0005">a</a>" ] ] ] "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/ajd.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. 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Diaz" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jpeds.2005.12.059" "Revista" => array:4 [ "tituloSerie" => "J Pediatr" "fecha" => "2016" "paginaInicial" => "17" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16860119" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack405583" "titulo" => "Acknowledgments" "texto" => "We would like to thank the Incontinentia Pigmenti Genetic Biobank (<a class="elsevierStyleInterRef" target="_blank" id="intr0010" href="http://www/">www</a>. igb. cnr. it/ ipgb), which forms part of the Biobanking and BioMolecular Resources Research Infrastructure-European Research Infrastructure Consortium (BBRMI-ERIC), and the CNR-DSB Flagship Project InterOmics.We also thank Rosalía Gumina F, Managing Director of the Library at the Instituto Autónomo Hospital Universitario de Los Andes, Universidad de Los Andes." "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000011000000004/v1_201905070820/S1578219019301015/v1_201905070820/en/main.assets" "Apartado" => array:4 [ "identificador" => "6151" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Practical Dermatology" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000011000000004/v1_201905070820/S1578219019301015/v1_201905070820/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219019301015?idApp=UINPBA000044"]

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Practical Dermatology

Incontinentia Pigmenti

Incontinencia pigmenti

F. Cammarata-Scalisia,

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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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              "identificador" => "abst0005"
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        1 => array:2 [
          "identificador" => "xpalclavsec1107568"
          "titulo" => "Keywords"
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          "titulo" => "Resumen"
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            0 => array:1 [
              "identificador" => "abst0010"
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        3 => array:2 [
          "identificador" => "xpalclavsec1107569"
          "titulo" => "Palabras clave"
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        4 => array:2 [
          "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"
        ]
        7 => array:2 [
          "identificador" => "sec0020"
          "titulo" => "Etiology and Pathogenesis"
        ]
        8 => array:2 [
          "identificador" => "sec0025"
          "titulo" => "Differential Diagnosis"
        ]
        9 => array:2 [
          "identificador" => "sec0030"
          "titulo" => "Conclusions and Genetic Counseling"
        ]
        10 => array:2 [
          "identificador" => "sec0035"
          "titulo" => "Conflicts of Interest"
        ]
        11 => array:2 [
          "identificador" => "xack405583"
          "titulo" => "Acknowledgments"
        ]
        12 => array:1 [
          "titulo" => "References"
        ]
      ]
    ]
    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2018-05-04"
    "fechaAceptado" => "2018-10-13"
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        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec1107568"
          "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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        0 => array:4 [
          "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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      0 => array:2 [
        "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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                0 => """
                  <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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        "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"
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        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:24 [
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              "identificador" => "bib0125"
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              "referencia" => array:1 [
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            1 => array:3 [
              "identificador" => "bib0130"
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
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                  "host" => array:1 [
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            2 => array:3 [
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            3 => array:3 [
              "identificador" => "bib0140"
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
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                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.3889/oamjms.2017.128"
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            4 => array:3 [
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                  "contribucion" => array:1 [
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                        0 => array:2 [
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                            0 => "F&#46; Fusco"
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                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1542/peds.2016-2950"
                      "Revista" => array:5 [
                        "tituloSerie" => "Pediatrics&#46;"
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                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28794079"
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            5 => array:3 [
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            6 => array:3 [
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
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                        0 => array:2 [
                          "etal" => true
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                            0 => "A&#46; Smahi"
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            7 => array:3 [
              "identificador" => "bib0160"
              "etiqueta" => "8"
              "referencia" => array:1 [
                0 => array:1 [
                  "referenciaCompleta" => "Scheuerle AE&#44; Ursini MV&#46; Incontinentia Pigmenti&#46; In&#58; Adam MP&#44; Ardinger HH&#44; Pagon RA&#44; Wallace SE&#44; editores&#46; GeneReviews<span class="elsevierStyleSup">&#174;</span>&#46; Seattle &#40;WA&#41;&#58; University of Washington&#44; Seattle&#59; 1993-2018&#46;"
                ]
              ]
            ]
            8 => array:3 [
              "identificador" => "bib0165"
              "etiqueta" => "9"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "A 6-month-old girl with Incontinentia Pigmenti presenting as status epilepticus"
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                        0 => array:2 [
                          "etal" => false
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                            0 => "M&#46;Y&#46; Seo"
                            1 => "S&#46;J&#46; You"
                            2 => "S&#46;H&#46; Kim"
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                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.14581/jer.17019"
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                        "tituloSerie" => "J Epilepsy Res&#46;"
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                        "volumen" => "7"
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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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Article information

ISSN: 15782190
Original language: English

DOI:10.1016/j.adengl.2019.03.009

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