Juvenile hyaline fibromatosis (JHF).

The juvenile hyaline fibromatosis (JHF) is a rare tumorous autosomal recess ive disease of the connective tissue. The etiopathogenesis of the disease i s unknown. Typical diagnostic criteria are multiple hyaline subcutaneous fi broma, filamentous tumors of the skin, gingival hypertrophy, muscle contrac tures of the extremities and multiple osteolytic bone destructions. Involve ment of visceral organs is not described. Mental development and life expec tancy are normal. Until today, no causal treatment of the JHF exists. Surgi cal excision of the dermal tumors is indicated for functional and aesthetic improvement. Complete excision should be executed in the early phase of th eir development. This has shown the best functional and aesthetic results, especially in facial areas. The progress of the disease is individual. Due to frequent recurrencies, repeated resections and revisions may be necessar y. Interdisciplinal counseling and therapy, particularly physiotherapy, may partially improve the state of function.