Anais Brasileiros de Dermatologia
Print version ISSN 0365-0596
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. http://dx.doi.org/10.1590/S0365-05962010000300013.
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.