Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis
AUTOR(ES)
Marques, Gabriela Franco, Tonello, Claudio Sampieri, Sousa, Juliana Martins Prazeres
FONTE
An. Bras. Dermatol.
DATA DE PUBLICAÇÃO
2014-06
RESUMO
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.
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