Gingival hyperplasia associated with juvenile hyaline fibromatosis: a case report and review of the literature.

摘要

"Molluscum fibrosum," a variant of the neurofibroma-tosis disorders, was first described by Murray in 1873. However, in 1973 it was Kitano et al who renamed the disease "juvenile hyaline fibromatosis" (JHF). There have been approximately 70 cases of JHF reported in the literature to date. JHF is a rare autosomal dominant systemic disease of the connective tissue, typically presenting in childhood. In 2002, Rahman et al performed a genetic analysis of 2 families with JHF and linked JHF to a mutation on chromosome 4q21. The exact underlying pathogenesis of JHF is yet to be identified, but some investigators have proposed it results from an abnor- mality in collagen or the result of defective glycos-aminoglycan formation.