Document Detail


Juvenile hyaline fibromatosis: a case report and review of literature.
MedLine Citation:
PMID:  19365310     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Sandra Lúcia Ribeiro; Erilane L Guedes; Valeria Botan; Alessandra Barbosa; Ernani J Freitas
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Acta reumatológica portuguesa     Volume:  34     ISSN:  0303-464X     ISO Abbreviation:  Acta Reumatol Port     Publication Date:    2009 Jan-Mar
Date Detail:
Created Date:  2009-04-14     Completed Date:  2009-07-07     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0431702     Medline TA:  Acta Reumatol Port     Country:  Portugal    
Other Details:
Languages:  eng     Pagination:  128-33     Citation Subset:  IM    
Affiliation:
Department of Rheumatology, Getúlio Vargas University Hospital (HUGV)/Health Science Faculty of Amazon Federal University (UFAM), Manaus, Brazil. sandraeuzebio@vivax.com.br
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MeSH Terms
Descriptor/Qualifier:
Fibroma / diagnosis*
Humans
Hyalin*
Infant
Male

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