Incontinentia pigmenti or Bloch-Sulzberger syndrome: A rare X-linked genodermatosis

Incontinentia pigmenti is a rare x-linked genodermatosis that affects mainly female neonates. the first manifestation occurs in the early neonatal period and progresses through four stages: vesicular,verruciform,hyperpigmented and hypopigmented. clinical features also manifest themselves through changes in the teeth,eyes,hair,central nervous system,bone structures,skeletal musculature and immune system. the authors report the case of a patient with cutaneous lesions and histological findings that are compatible with the vesicular stage,emphasizing the importance of early diagnosis and appropriate therapeutic management. © 2014 by anais brasileiros de dermatologia.