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 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 32 Issue: 6
  • Publication Date: 2005
  • Doi Number: 10.1111/j.0303-6987.2005.00341.x
  • Journal Name: JOURNAL OF CUTANEOUS PATHOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.438-440
  • Karadeniz Technical University Affiliated: Yes

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.