title: Therapy of noninfectious granulomatous skin diseases with fumaric acid esters.
abstract: BACKGROUND
  Noninfectious granulomatous skin diseases are inflammatory disorders of unknown aetiology which are often recalcitrant to common anti-inflammatory treatment regimens. Recently, in several case reports, fumaric acid esters (FAE) have proved beneficial in granulomatous skin diseases, but studies on a larger collection of consecutive patients have not yet been performed.
  
  
  OBJECTIVE
  To investigate the therapeutic efficacy of FAE for the treatment of granulomatous skin diseases.
  
  
  METHODS
  The therapeutic efficacy and side-effects of FAE were analysed retrospectively in 32 patients with disseminated granuloma annulare (n = 13), annular elastolytic giant cell granuloma (n = 3), sarcoidosis (n = 11), necrobiosis lipoidica (n = 4), or granulomatous cheilitis (n = 1).
  
  
  RESULTS
  Three patients discontinued treatment within 4 weeks because of side-effects. Of the remaining 29 patients, 18 patients responded to treatment with FAE. Marked improvement or complete clearance was seen in seven patients. We observed a slight to moderate improvement in 11 patients, and 11 patients did not respond. In patients showing a complete remission, the maximum effect was observed after 8.5 months (SD +/-6 months, range 3-20 months). In two patients with systemic sarcoidosis, the pulmonary changes improved in parallel with the skin. Side-effects were usually mild and resolved spontaneously upon dose reduction or discontinuation of the therapy.
  
  
  CONCLUSIONS
  The data presented here indicate that FAE may be considered for the treatment of recalcitrant granulomatous skin disease.
authors.affiliation: Department of Dermatology and Allergology, Hannover Medical University, Ricklinger Strasse 5, 30449 Hannover, Germany. breuer.kristine@mh-hannover.de
authors.forename: K
authors.initials: K
authors.lastname: Breuer
authors.forename: R
authors.initials: R
authors.lastname: Gutzmer
authors.forename: B
authors.initials: B
authors.lastname: Völker
authors.forename: A
authors.initials: A
authors.lastname: Kapp
authors.forename: T
authors.initials: T
authors.lastname: Werfel
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002613:Cheilitis / Q000188:drug therapy; D004334:Drug Administration Schedule; D004952:Esters; D005260:Female; D005650:Fumarates / Q000008:administration & dosage* / Q000627:therapeutic use; D016460:Granuloma Annulare / Q000188:drug therapy; D006101:Granuloma, Giant Cell / Q000188:drug therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D009335:Necrobiosis Lipoidica / Q000188:drug therapy; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000188:drug therapy; D012871:Skin Diseases / Q000188:drug therapy*
chemical_list: D004952:Esters; D005650:Fumarates; C032005:fumaric acid