Case and Research Letter
Is Incontinentia Pigmenti More Serious in Males? A Report of 2 Cases
Incontinencia pigmenti: ¿es una entidad más grave en los varones? Reporte de 2 casos
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C) Epidermis: espongiosis con formación de vesículas espongióticas intradérmicas y exocitosis de numerosos eosinófilos. Células disqueratósicas con citoplasma hialino. Dermis con edema e infiltrado inflamatorio superficial mononuclear con eosinófilos y melanófagos.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "V. Vezzaro, M. de María, L. Lucas, A. Acosta" "autores" => array:4 [ 0 => array:2 [ "nombre" => "V." "apellidos" => "Vezzaro" ] 1 => array:2 [ "nombre" => "M." "apellidos" => "de María" ] 2 => array:2 [ "nombre" => "L." "apellidos" => "Lucas" ] 3 => array:2 [ "nombre" => "A." 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"apellidos" => "Vezzaro" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:4 [ "nombre" => "M." "apellidos" => "De María" "email" => array:1 [ 0 => "manudemaria12@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:3 [ "nombre" => "L." "apellidos" => "Lucas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "A." "apellidos" => "Acosta" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Departamento de Neonatología – Centro Hospitalario Pereira Rossell, Universidad de la República, Montevideo, Uruguay" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Cátedra de Dermatología, Centro Hospitalario Pereira Rossell, Universidad de la República, Montevideo, Uruguay" "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: ¿es una entidad más grave en los varones? Reporte de 2 casos" ] ] "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" => 377 "Ancho" => 1305 "Tamanyo" => 135627 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Epidermal acanthosis. Subcorneal vesicle with eosinophils. B, Epidermal acanthosis. Dermis with eosinophils. C, Perivascular dermal inflammatory infiltrate with eosinophils.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Incontinentia pigmenti (IP) is a rare, multisystemic, genodermatosis (0.7 cases per 100<span class="elsevierStyleHsp" style=""></span>000 births).<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">With a highly variable phenotype, its most common clinical manifestations are dermatologic, neurologic, ocular, and dental abnormalities.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Skin changes, a marker of the disease, appear early and characteristically follow the lines of Blaschko in all their stages.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Four sequential stages have been described for typical IP lesions: vesicles (at birth or shortly afterwards), verrucous lesions, hyperpigmentation, and hypopigmentation.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Extracutaneous involvement (neurologic and ocular alterations) determines functional prognosis and leads to irreversible sequelae linked to severe disease.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1–7</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">IP is an X-linked dominant disorder caused by a mutation in the <span class="elsevierStyleItalic">NEMO</span> gene. Between 65% and 75% of cases are caused by sporadic mutations.</p><p id="par0035" class="elsevierStylePara elsevierViewall">IP with an X-linked dominant inheritance pattern is usually lethal in male fetuses, with very few survivors reported.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,5</span></a> The International Incontinentia Pigmenti Consortium has proposed 3 mechanisms to explain why some males survive: <span class="elsevierStyleItalic">1</span>) presence of a concomitant 47,XXY karyotype (Klinefelter syndrome), <span class="elsevierStyleItalic">2</span>) somatic mosaicism (46,XY/47,XXY), which can only be demonstrated by fluorescence in situ hybridization, and <span class="elsevierStyleItalic">3</span>) hypomorphic mutations resulting in mild IP. Somatic mosaicism is the most common mechanism.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">We describe 2 cases of IP in male neonates and review the diagnostic criteria, etiology, pathogenesis, clinical manifestations, and complications of this rare disease.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The first case involved a 4-day-old boy born to a healthy 37-year-old woman following a well-controlled, complication-free pregnancy. The woman had 3 other children, all healthy, from 3 pregnancies. The boy had been born at term (37 weeks) by vaginal delivery. At birth, he had a series of vesiculobullous lesions with yellowish fluid along the lines of Blaschko on the inner aspect of the left leg.</p><p id="par0050" class="elsevierStylePara elsevierViewall">The lesions evolved into verrucous and subsequently hyperpigmented lesions (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A and B).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0055" class="elsevierStylePara elsevierViewall">Histopathologic features were consistent with early-stage IP (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">The boy had a 46,XY normal karyotype, and no abnormalities were observed in the fundus examination or the transfontanellar ultrasound. He progressed favorably and was discharged at 15 days of life.</p><p id="par0065" class="elsevierStylePara elsevierViewall">The second case involved a 9-day-old boy born to a healthy 36-year-old mother with a child from another pregnancy. The pregnancy had been well controlled and free of complications. The boy had been born by vaginal delivery at term. At birth, he had vesiculobullous lesions with clear fluid on the right leg; the lesions followed a linear distribution in the dorsal popliteal region. He was discharged at 48<span class="elsevierStyleHsp" style=""></span>h of life without paraclinical tests.</p><p id="par0070" class="elsevierStylePara elsevierViewall">When the boy was 9 days old, the pediatrician examined the lesions, which were verrucous by this stage, and established a presumptive diagnosis of IP (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A). The rest of the physical examination was normal.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0075" class="elsevierStylePara elsevierViewall">The area affected by the lesions became smaller, leaving a series of hyperpigmented lesions limited to the popliteal area (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B).</p><p id="par0080" class="elsevierStylePara elsevierViewall">The histopathology study showed IP in the vesicular phase (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>C).</p><p id="par0085" class="elsevierStylePara elsevierViewall">The boy had a 46,XY normal karyotype, and the fundus examination and transfontanellar ultrasound showed no alterations. He progressed well and the skin lesions disappeared spontaneously.</p><p id="par0090" class="elsevierStylePara elsevierViewall">IP is a rare X-linked genodermatosis.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,3,4,6–9</span></a> It is seldom diagnosed in male neonates, as its mode of inheritance is normally associated with fetal death.</p><p id="par0095" class="elsevierStylePara elsevierViewall">Few cases of surviving males have been reported worldwide.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6,9</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">The presumptive diagnosis is clinical and based on observation of early skin lesions.</p><p id="par0105" class="elsevierStylePara elsevierViewall">Both patients described in this series had vesiculobullous lesions (stage 1) along the lines of Blaschko at birth and subsequently developed verrucous lesions and hyperpigmentation.</p><p id="par0110" class="elsevierStylePara elsevierViewall">Typical skin lesions are considered major criteria for the diagnosis of IP.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">According to Rosser,<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> 2 or more major diagnostic criteria or 1 major and 1 minor criterion are required (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>) to confirm diagnosis when there is no evidence of IP in a first-degree female relative and when molecular diagnosis (detection of <span class="elsevierStyleItalic">NEMO</span> mutation) is not available (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0120" class="elsevierStylePara elsevierViewall">The 2 patients in this series had typical skin IP skin changes (major criterion) and typical histopathologic findings (minor criterion).</p><p id="par0125" class="elsevierStylePara elsevierViewall">Although IP is generally considered lethal in males, there have been some survivors. Proposed mechanisms to explain the small number of survivors reported are the concomitant presence of the 47,XXY karyotype (Klinefelter syndrome) and somatic mosaicism.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6,8</span></a> In both our cases, the karyotype was normal. Based on frequency, somatic mosaicism could explain why these patients survived.</p><p id="par0130" class="elsevierStylePara elsevierViewall">The main entities to be considered in the differential diagnosis are other skin diseases that present with vesiculobullous lesions. Because of their frequency and severity, infectious causes must be ruled out.</p><p id="par0135" class="elsevierStylePara elsevierViewall">In the second case in this series, the patient was not diagnosed at birth, despite the presence of characteristic IP lesions. IP is rare and therefore there is a low index of suspicion among physicians. It is probably underdiagnosed or misdiagnosed in patients with mild forms.</p><p id="par0140" class="elsevierStylePara elsevierViewall">There are clinical cases indicating that mosaicism is common in male patients with IP who do not develop multisystemic disease and who have skin changes that tend to disappear, as in our patients.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6,8</span></a></p><p id="par0145" class="elsevierStylePara elsevierViewall">Central nervous system involvement and eye abnormalities are the main determinants of morbidity and mortality in IP.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,2,5,8</span></a> Because these alterations can appear with time, multidisciplinary follow-up and genetic counseling are important.</p><p id="par0150" class="elsevierStylePara elsevierViewall">This study was approved by the ethics committee at Centro Hospitalario Pereira Rossell. Consent for publication was obtained from the patients’ parents.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0155" class="elsevierStylePara elsevierViewall">The authors declare no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 687 "Ancho" => 1256 "Tamanyo" => 92251 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Vesiculobullous lesions with a diameter of 2 to 3<span class="elsevierStyleHsp" style=""></span>mm on the inner aspect of the left leg along the lines of Blaschko. B, Verrucous, crusty lesions with hyperpigmentation.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 377 "Ancho" => 1305 "Tamanyo" => 135627 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Epidermal acanthosis. Subcorneal vesicle with eosinophils. B, Epidermal acanthosis. Dermis with eosinophils. C, Perivascular dermal inflammatory infiltrate with eosinophils.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 368 "Ancho" => 1305 "Tamanyo" => 73730 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A and B, Verrucous (stage 2) lesions along the lines of Blaschko. C, Epidermis: spongiosis with intradermal spongiotic vesicles and exocytosis of numerous eosinophils. Dyskeratotic cells with hyaline cytoplasm. Dermis with edema and mononuclear superficial inflammatory infiltrate with eosinophils and melanophages.</p>" ] ] 3 => 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:2 [ "leyenda" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">In all cases eosinophilia or skewed X chromosome inactivation support a diagnosis.</p>" "tablatextoimagen" => array:1 [ 0 => array:1 [ "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="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Major criteria \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Minor criteria \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Typical neonatal rash consisting of vesicles and erythema (stage 1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dental anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Verrucous papules or plaques along the lines of Blaschko (stage 2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Central nervous system anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Typical hyperpigmentation along the lines of Blaschko (stage 3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Eye anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Linear, atrophic, hairless lesions on extremities (stage 4) or scarring alopecia of the vertex \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mammary gland and/or nipple anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Palate anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Hair anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Nail anomalies \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Multiple miscarriages \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Typical skin histopathologic findings \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Diagnostic Criteria for Incontinentia Pigmenti.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Multidisciplinary consensus recommendations from a European network for the diagnosis and practical management of patients with incontinentia pigmenti" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "C. 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