Juvenile Hyaline Fibromatosis: A 10-year Follow-up


Baltacıoğlu E. , Güzeldemir Akçakanat E., Sukuroglu E., Yıldız K. , Yuva P., Aydin G. , ...More

INDIAN JOURNAL OF DERMATOLOGY, vol.62, no.2, pp.210-212, 2017 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Editorial Material
  • Volume: 62 Issue: 2
  • Publication Date: 2017
  • Doi Number: 10.4103/ijd.ijd_166_16
  • Title of Journal : INDIAN JOURNAL OF DERMATOLOGY
  • Page Numbers: pp.210-212

Abstract

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.