Juvenile hyaline fibromatosis of the mandible with bone involvement: Report of a rare case

Juvenile hyaline fibromatosis (JHF) is a rare autosomal-recessive hereditary disease, characterized by gingival hypertrophy, flexion contractures of joints, bone lesions, hyaline deposition in the extracellular spaces of the dermis and soft tissues, stunted growth, and skin lesions such as multiple nodules, tumors and pink, pearly papules. No case of JHF with a mandibular bone involvement, exists in the literature. Bone involvement in JHF is an uncommon finding and distinct solitary lesions in the calvarial bones has been reported by some authors. A 21-year-old male patient was referred to Diyarbakir Military Hospital, Department of Dental Service. Clinical findings were consistent with a solid alveolar mass in the right mandibular premolar-molar region and displaced right mandibular molar teeth. Orthopantomographic examination showed impaction of all lower right molars in a mixed radioopaque/radiolucent area. Microscopically, increased nodular connective tissue was seen under the lobulated mucosal surfaces of the resected area. The case presented here had a localized fibrous proliferation that infiltrated bone trabeculae and caused displacement of teeth. Juvenile hyaline fibromatosis should be considered in the differential diagnosis with the other intraosseous radiolucent-patchy opaque lesions of jaw bones. Based on the clinical and histopathological findings, a diagnosis of JHF was made. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:e59-e63)