The systemic hyalinoses are rare autosomal recessive disorders, with multiorgan deposition of amorphous hyaline material. The conditions have a wide spectrum of expressivity, varying from infantile generalized forms with early death, to localized juvenile forms with prolonged survival , Clinical features include skin lesions, gingival hyperplasi,joint contractures and bone involvement--typically diffuse osteopenia and small osteolytic lesions .
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