Juvenile non-hyaline fibromatosis: juvenile hyaline fibromatosis without prominent hyaline changes
Authors: Anadolu, Rana Yavuzer1; Oskay, Tugˇba2; Özsoy, Nesrin3; Erdem, Cengizhan1
Source: Journal of Cutaneous Pathology, Volume 32, Number 3, March 2005 , pp. 235-239(5)
Publisher: Blackwell Publishing
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Abstract:
: Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease of the connective tissue. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, osteolytic bone lesions and flexion contractures of the large joints. Here, we report a 14-year-old girl with characteristic clinical features of JHF with early fatal outcome. Dermatopathologic examination of the early lesions however constantly lacked the so-called hyalin changes in multiple skin biopsies.According to our experience; dermatopathological features of this entitity is not often and always consists of classical hyalinisation. Only larger lesions with long duration should expected to be exhibiting those features. Therefore we suggest that; JHF may often present itself as Juvenil Non-Hyaline Fibromatosis: JHF without prominent hyaline changes. And thus this fact should not change the actual diagnosis and prognostic implications. Anadolu RY, Oskay T, Özsoy N, Erdem C. Juvenile non-hyaline fibromatosis: juvenile hyaline fibromatosis without prominent hyaline changes.Document Type: Research article
DOI: 10.1111/j.0303-6987.2005.00275.x
Affiliations: 1: Department of Dermatology, Ankara University Medical School, 2: Department of Dermatology, Bayındır Medical Centre, and 3: Department of Biology, Ankara University Faculty of Science, Ankara, Turkey
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