Incontinentia pigmenti or Bloch-Sulzberger syndrome: a rare X-linked genodermatosis
An. bras. dermatol
; 89(3): 486-489, May-Jun/2014. graf
Article
in En
| LILACS
| ID: lil-711602
Responsible library:
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.
Key words
Full text:
1
Index:
LILACS
Main subject:
Incontinentia Pigmenti
/
Genetic Diseases, X-Linked
/
Rare Diseases
Type of study:
Screening_studies
Limits:
Female
/
Humans
/
Infant
Language:
En
Journal:
An. bras. dermatol
Journal subject:
DERMATOLOGIA
Year:
2014
Type:
Article