Juvenile non-hyaline fibromatosis: juvenile hyaline fibromatosis without prominent hyaline changes

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Anadolu R., Oskay T., Ozsoy N., Erdem C.

JOURNAL OF CUTANEOUS PATHOLOGY, vol.32, no.3, pp.235-239, 2005 (SCI-Expanded, Scopus)

• Publication Type: Article / Article
• Volume: 32 Issue: 3
• Publication Date: 2005
• Doi Number: 10.1111/j.0303-6987.2005.00275.x
• Journal Name: JOURNAL OF CUTANEOUS PATHOLOGY
• Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
• Page Numbers: pp.235-239
• Ankara University Affiliated: Yes

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.