A Case of Juvenile Hyalin Fibromatosis / 대한피부과학회지

ABSTRACT

Juvenile Hyalin Fibromatosis is a rare congenital disease that is characterized by large tumors, especially on the scalp, small whitish nodules, hypertrophy of the gingiva, and flexural contractures of the large joints of the limbs. A review of the world literature disclosed 13 similar cases, under various names. This case is the 14th case in the literature and the first case in Korea. A 3 year old girl had multiple large tumors and nodules on various parts of the bodis, hypertrophic gingiva, and flexural contracture of the elbow and knee joints. The histopathologic features of the tumor were characteristic of Juvenile Hyalin Fibromatosis. The tumor cells were embedded in an amorphous eosinophilic ground substance. Diagnosis was confirmed by clinical features and histopathological findings.