Juvenile hyaline fibromatosis.
Pediatr Dermatol
; 18(5): 400-2, 2001.
Article
em En
| MEDLINE
| ID: mdl-11737684
Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Cutâneas
/
Fibroma
/
Fibromatose Gengival
Limite:
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Pediatr Dermatol
Ano de publicação:
2001
Tipo de documento:
Article
País de afiliação:
Argentina
País de publicação:
Estados Unidos