Juvenile hyalin fibromatozis:A 10-year follow-up


Baltacıoğlu E., Güzeldemir Akçakanat E., Şüküroğlu E., Yıldız K., Yuva P., Aydın G., ...Daha Fazla

INDIAN JOURNAL OF DERMATOLOGY, cilt.62, sa.2, ss.210-212, 2017 (SCI-Expanded)

  • Yayın Türü: Makale / Vaka Takdimi
  • Cilt numarası: 62 Sayı: 2
  • Basım Tarihi: 2017
  • Dergi Adı: INDIAN JOURNAL OF DERMATOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.210-212
  • Karadeniz Teknik Üniversitesi Adresli: Evet

Özet

Juvenile hyaline fibromatosis (JHF) is a rare hereditary disease with an autosomal recessive transmission. JHF is characterized by papulonodular skin lesions, osteolytic bone lesions, flexural joint contractures, and gingival hyperplasia and usually diagnosed in infancy or early childhood. JHF is thought to be a disorder of collagen metabolism and characterized by homogenous amorphous eosinophilic material and fibrous tissue. We report the case of a 14-year-old male child with multiple papulonodular skin lesions, progressive flexion contractures of joints, and severe gingival hyperplasia, with a 10-year follow-up. Although the lesions were totally removed thrice during the last 10 years, they recurred rigorously.