Juvenile non-hyaline fibromatosis: juvenile hyaline fibromatosis without prominent hyaline changes

Anadolu R., Oskay T., Ozsoy N., Erdem C.

JOURNAL OF CUTANEOUS PATHOLOGY, cilt.32, sa.3, ss.235-239, 2005 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 32 Sayı: 3
  • Basım Tarihi: 2005
  • Doi Numarası: 10.1111/j.0303-6987.2005.00275.x
  • Dergi Adı: JOURNAL OF CUTANEOUS PATHOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.235-239
  • Ankara Üniversitesi Adresli: Evet

Özet

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease of the connective tissue. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, osteolytic bone lesions and flexion contractures of the large joints. Here, we report a 14-year-old girl with characteristic clinical features of JHF with early fatal outcome. Dermatopathologic examination of the early lesions however constantly lacked the so-called hyalin changes in multiple skin biopsies.