Juvenile hyaline fibromatosis: a case report


Karacal N. , GULCELIK N., Yildiz K. , Mungan S. , KUTLU N.

JOURNAL OF CUTANEOUS PATHOLOGY, vol.32, no.6, pp.438-440, 2005 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 32 Issue: 6
  • Publication Date: 2005
  • Doi Number: 10.1111/j.0303-6987.2005.00341.x
  • Title of Journal : JOURNAL OF CUTANEOUS PATHOLOGY
  • Page Numbers: pp.438-440

Abstract

Juvenile hyaline fibromatosis ( JHF ) is a rare autosomal recessive disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and bone lesions. The skin lesions may consist of multiple large tumors, commonly on the scalp and around the neck, and small pearly, pink papules and plaques on the trunk, chin, ears, and around the nostrils. Here, we report a 2-year-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hyperplasia. The lesions were totally excised and clinicopathological diagnosis was JHF.