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Anais Brasileiros de Dermatologia

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PEREIRA, Marcela A. C. et al. X-linked incontinentia pigmenti or Bloch-Sulzberger syndrome: a case report. An. Bras. Dermatol. [online]. 2010, vol.85, n.3, pp.372-375. ISSN 0365-0596.

Incontinentia pigmenti is a rare X-linked genodermatosis that affects mainly female neonates. Skin manifestations are the most common and occur in four quite distinct phases. A female infant presented vesiculobullous lesions on trunk and limbs, and a verrucous lesion on the right palm. Biopsy revealed eosinophil exocytosis and pigment incontinence, confirming the clinical hypothesis. Although uncommon, incontinentia pigmenti should be taken into consideration as a possible differential diagnosis when vesiculobullous and verrucous lesions are present in childhood.

Keywords : Genetics; Incontinentia pigmenti; X chromosome.

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