title: Juvenile hyaline fibromatosis: a case report.
abstract: 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.
authors.affiliation: Faculty of Medicine, Department of Otorhinolaryngology, Baskent University, Ankara, Turkey. fcaylakli@yahoo.com
authors.forename: Fatma
authors.initials: F
authors.lastname: Caylakli
authors.forename: Ozcan
authors.initials: O
authors.lastname: Cakmak
authors.forename: Deniz
authors.initials: D
authors.lastname: Seckin
authors.forename: Fazilet
authors.initials: F
authors.lastname: Kayaselcuk
authors.forename: Beyhan
authors.initials: B
authors.lastname: Demirhan
authors.forename: Levent Naci
authors.initials: LN
authors.lastname: Ozluoglu
mesh_terms: D002648:Child; D005260:Female; D005350:Fibroma / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D006818:Hyalin / Q000378:metabolism; D009669:Nose Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D012225:Rhinoplasty; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery