Abstract
– Juvenile hyaline fibromatosis (JHF) is a mesenchimal dysplasia of autosomal recessive inheritance with onset in infancy or early childhood. So far, around 65 cases of JHF have seen described. It is characterized by skin lesions, gingival hypertrophy, flexion contractures of the large joints and bone lesions. Skin lesions may consist of multiple large tumors, commonly on the scalp and around the neck, and small pearly, pink papules and plaques on the trunk, chin, ears and around the nostrils. JHF is characterized by an aberrant synthesis of collagen, with is then deposited as hyaline material in the connective tissues of the skin, in the gingival and less commonly in the bones and joints. We present a 15 month-old girl with severe joint contractures and skin lesions typical of JHF.
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