JUVENILE HYALINE FIBROMATOSIS (A CASE-REP ORT)

We report the case of a child of unrelated parents with Juvenile Hyali ne Fibromatosis (JHF) a rare disease inherited as a recessive autosoma l trait, consisting of skin lesions, gingival hypertrophy, flexion con tractures of the joints and osteolytic defects. In our patient a pseud o-Hurler facial dysmorphism and a hypertrichosis, not so typical findi ngs, were also present. To our knowledge, our case is the first report of JHF in Italy.