Juvenile hyaline fibromatosis: 2 twin brothers affected
Habibeddine, S.; Khadir, K.; Azzouzi, S.; Skalli, S.; Lakhdar, H.
Annales de Dermatologie et de Venereologie 130(1 Pt 1): 43-46
2003
ISSN/ISBN: 0151-9638 PMID: 12605157 Document Number: 565792
Introduction. Juvenile hyaline fibromatosis is a rare hereditary genetic disease characterized by a cutaneous tumoral fibroblastic proliferation with joint involvement. Case-report. We report the case of 2 homozygous twin brothers both presenting a diffuse and invalidating form of juvenile hyaline fibromatosis. The 2 boys' born of a twin pregnancy' presented at the age of 2 months a clinical picture made up by the classic association: infiltrated papulo-nodules, gingival hyperplasia and joint contracture. The diagnosis of juvenile hyaline fibromatosis suggested clinically was confirmed by the histological study of the skin. Discussion. These observations of juvenile hyaline fibromatosis's are particular in the involvement of 2 twin brothers, which to our knowledge has never been reported, the resemblance of the clinical features, and the severity of the functional handicap.