Juvenile hyaline fibromatosis: a case report

Juvenile hyaline fibromatosis (JHF) is a rare disease with autosomal recessive inheritance. It is characterized by papulonodular skin lesions which are painless but cosmetically unacceptable, and often disturb normal joint function. The nose, chin, ears, scalp, back, and knees are the most common affected sites. In some cases, gingival hypertrophy, flexion contractures of the large joints, osteolytic bone lesions, and stunted growth may be associated with skin lesions. We report a 9-year-old girl presented with a soft, painless, slow growing, tumoral mass on the supratip of her nose. The lesion was totally excised with open rhinoplasty approach and histopathological diagnosis was JHF.