Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis
An. bras. dermatol
; 89(3): 486-489, May-Jun/2014. graf
Article
em En
| LILACS
| ID: lil-711602
Biblioteca responsável:
BR1.1
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Índice:
LILACS
Assunto principal:
Incontinência Pigmentar
/
Doenças Genéticas Ligadas ao Cromossomo X
/
Doenças Raras
Tipo de estudo:
Screening_studies
Limite:
Female
/
Humans
/
Infant
Idioma:
En
Revista:
An. bras. dermatol
Assunto da revista:
DERMATOLOGIA
Ano de publicação:
2014
Tipo de documento:
Article