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Juvenile hyaline fibromatosis: a case report and review of literature.
Ribeiro, Sandra Lúcia; Guedes, Erilane L; Botan, Valeria; Barbosa, Alessandra; Freitas, Ernani J.
Afiliação
  • Ribeiro SL; Department of Rheumatology, Getúlio Vargas University Hospital (HUGV)/Health Science Faculty of Amazon Federal University (UFAM), Manaus, Brazil. sandraeuzebio@vivax.com.br
Acta Reumatol Port ; 34(1): 128-33, 2009.
Article em En | MEDLINE | ID: mdl-19365310
ABSTRACT
Juvenile hyaline fibromatosis (JHF) is a rare disease with autosomal recessive inheritance that occurs mainly in childhood and is characterized by the deposition of amorphous hyaline material in the skin and other organs. There are approximately 70 cases reported in the literature. Herein we describe the case of a 14-month-old boy with multiple cutaneous nodules around small and large joints, papulous skin lesions, hyperpigmented plaques and nodules in the perianal region, flexion contractures and stiffness of joints and diffuse osteoporosis. Symptoms were present since the second month of life. Histopathologic studies of joint nodulations demonstrated the presence of hyaline material, confirming the diagnosis of juvenile hyaline fibromatosis.
Assuntos
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Base de dados: MEDLINE Assunto principal: Fibroma / Hialina Idioma: En Ano de publicação: 2009 Tipo de documento: Article
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Base de dados: MEDLINE Assunto principal: Fibroma / Hialina Idioma: En Ano de publicação: 2009 Tipo de documento: Article