JUVENILE HYALINE FIBROMATOSIS

Abstract

Juvenile hyaline fibromatosis is a rare, autosomal recessive disease. We report a 4-year-old female born offirst-degree consanguineous marriage, presenting with gingival hyperplasia, nodular swellings involvingthe both pinna and right ankle joint. She had contractures of elbows, knees and shoulder joints, restrictingthe movements of these joints. Cutaneous examination revealed erythematous papules and plaquesinvolving the nape of neck and the perianal region. Histological examination revealed deposition of PASpositive amorphous eosinophilic hyaline material with scattered intervening fibroblast like cells resemblingchondroid cells. The patient was diagnosed as a case of Juvenile hyaline fibromatosis based oncharacteristic clinical and histopathological findings.