Incontinentia Pigmenti: an unusual and fast presentation

Abstract

Incontinentia pigmenti is a rare X-linked neuroectodermal dysplasia estimated to occur in approximately 1:50,000 births. It’s first clinical manifestations are unique cutaneous lesions comprehending four different stages that emerge throughout the first year of life and persist until adolescence. One third of these patients develop central nervous system and ocular manifestations, causing great disability. The authors report the case of a patient with vesicular cutaneous lesions within 5 days after birth, with a fast and uncommon evolution. Although rare, the early identification of this disorder can prompt thorough investigation of associated comorbidities and adequate familiar and medical assistance.