title: Reticular erythematous mucinosis syndrome: glycosaminoglycan synthesis by fibroblasts and abnormal response to interleukin-1 beta.
abstract: BACKGROUND
  Reticular erythematous mucinosis (REM) syndrome is characterized by an accumulation of mucin in the dermis.
  
  
  OBJECTIVE
  To elucidate the mechanism of mucin deposition, the response of REM syndrome fibroblasts to various exogenous factors was studied.
  
  
  METHODS
  REM and control fibroblasts were treated with potent modulators including tumor necrosis factor-alpha, basic fibroblast growth factor, interferon-gamma, transforming growth factor-beta 1 and interleukin-1 alpha (IL-1 alpha) and -4.
  
  
  RESULTS
  IL-1 beta failed to stimulate glycosaminoglycan synthesis in REM fibroblasts whereas it stimulated glycosaminoglycan synthesis up to 6-fold in control fibroblasts. The stimulation of glycosaminoglycan was caused largely by a comparable increase in hyaluronic acid.
  
  
  CONCLUSIONS
  These results suggest that the patient fibroblasts exhibit an abnormal response to stimulation by exogenous IL-1 beta and that IL- 1 beta may be involved in the abnormal hyaluronic acid metabolism in REM syndrome.
authors.affiliation: Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Izumi
authors.forename: S
authors.initials: S
authors.lastname: Tajima
authors.forename: R
authors.initials: R
authors.lastname: Harada
authors.forename: T
authors.initials: T
authors.lastname: Nishikawa
mesh_terms: D000328:Adult; D018929:Cell Culture Techniques; D004890:Erythema / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D005347:Fibroblasts / Q000187:drug effects* / Q000378:metabolism; D006025:Glycosaminoglycans / Q000032:analysis / Q000096:biosynthesis*; D006801:Humans; D007375:Interleukin-1 / Q000494:pharmacology / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D017520:Mucinoses / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D012016:Reference Values; D012871:Skin Diseases / Q000188:drug therapy* / Q000378:metabolism* / Q000473:pathology; D013577:Syndrome
chemical_list: D006025:Glycosaminoglycans; D007375:Interleukin-1