Juvenile hyaline fibromatosis.

Larralde, M; Santos-Muñoz, A; Calb, I; Magariños, C

Larralde, M; Santos-Muñoz, A; Calb, I; Magariños, C.

Afiliação

Larralde M; Pediatric Dermatology Division, Ramos Mejia Hospital, Buenos Aires, Argentina. cymluna@fmed.uba.ar

Pediatr Dermatol ; 18(5): 400-2, 2001.

Article em En | MEDLINE | ID: mdl-11737684

RESUMO

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.

Assuntos

Fibroma/patologia; Fibromatose Gengival/patologia; Neoplasias Cutâneas/patologia; Biópsia; Contratura/patologia; Fácies; Humanos; Lactente; Masculino; Microscopia Eletrônica

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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

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