title: Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone.
abstract: BACKGROUND
  The group of autoinflammatory syndromes associated with Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis are poorly defined and difficult to control with currently available treatment modalities.
  
  
  OBJECTIVE
  We describe a patient with PASH syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone.
  
  
  METHODS
  A review of the available literature to date about this group of autoinflammatory diseases was performed. We performed genetic analysis for PSTPIP1 mutations associated with PAPA syndrome.
  
  
  RESULTS
  A 22-year-old woman presented to our department with pyoderma gangrenosum, concomitant acne, and suppurative hidradenitis. She had previously been treated unsuccessfully with etanercept, adalimumab, fumaric acid and the IL-1 receptor antagonist (IL-1RA) anakinra without prolonged remission. Treatment with intravenous infliximab in combination with cyclosporine and dapsone lead to sudden and prolonged improvement of the clinical symptoms that we classified as PASH syndrome. We review the literature about this group of diseases and report the third case of PASH syndrome to date.
  
  
  CONCLUSIONS
  PASH syndrome and associated diseases should be considered whenever hidradenitis suppurativa is found in association with pyoderma gangrenosum. We provide a systematic overview about PASH syndrome and suggest a novel multimodal therapeutic regimen beyond isolated inhibition of TNF or IL-1.
authors.affiliation: Department of Dermatology, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Staub
authors.affiliation: Department of Dermatology and Venereology, Federal Academic Teaching Hospital, Feldkirch, Austria.
authors.forename: N
authors.initials: N
authors.lastname: Pfannschmidt
authors.affiliation: Department of Dermatology and Venereology, Federal Academic Teaching Hospital, Feldkirch, Austria.
authors.forename: R
authors.initials: R
authors.lastname: Strohal
authors.affiliation: Department of Dermatology and Allergy, Ludwig-Maximilian University, Munich, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Braun-Falco
authors.affiliation: Institute for Laboratory Medicine and Human Genetics, Singen, Germany.
authors.forename: P
authors.initials: P
authors.lastname: Lohse
authors.affiliation: Department of Dermatology, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Goerdt
authors.affiliation: Department of Dermatology, University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Leverkus
mesh_terms: D000152:Acne Vulgaris / Q000188:drug therapy*; D000890:Anti-Infective Agents / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use*; D003622:Dapsone / Q000627:therapeutic use*; D003879:Dermatologic Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D017497:Hidradenitis Suppurativa / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000069285:Infliximab / Q000627:therapeutic use*; D017511:Pyoderma Gangrenosum / Q000188:drug therapy*; D013577:Syndrome; D055815:Young Adult
chemical_list: D000890:Anti-Infective Agents; D003879:Dermatologic Agents; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003622:Dapsone; D000069285:Infliximab