Juvenile hyaline fibromatosis in one Turkish child


Ugras S., Akpolat N., Metin A.

TURKISH JOURNAL OF PEDIATRICS, vol.42, no.3, pp.264-266, 2000 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 42 Issue: 3
  • Publication Date: 2000
  • Title of Journal : TURKISH JOURNAL OF PEDIATRICS
  • Page Numbers: pp.264-266

Abstract

We describe a case of juvenile hyaline fibromatosis (JHF) in a Turkish child. Only about 40 cases of juvenile hyaline fibromatosis had been reported in English literature as of March 1998, and it had not been reported in English literature from Turkey as of November 1998. Juvenile hyaline fibromatosis characterized by multiple cutaneous masses is a rare hereditary disorder. This disease is usually found in children, and a malfunction of collagen synthesis is considered as the pathogenetic cause. In the presented case, light microscopy demonstrated an abundance of a homogeneous, amorphous, eosinophilic extracellular matrix in which fibroblasts were embedded. Well-formed collagen fibers could not be demonstrated with Gieson's method or with reticulin preparation. The hayalin material periodic acid-Schiff-positive and diastase-resistant, whereas the Congo red method was negative. Immunohistochemically, the spindle-shaped cells were actin (smooth muscle) negative.