title: [Severe acne in autoinflammatory diseases].
abstract: BACKGROUND
  In recent years, acne has been described as a symptom of autoinflammatory diseases, such as PASH (pyoderma gangrenosum - PG, acne and suppurative hidradenitis - SH) and PAPASH (PG, acne, pyogenic arthritis, and SH). The pathogenesis of autoinflammatory diseases is not fully understood; however, based on the possible involvement of IL-1β, the recombinant human interleukin-1 receptor antagonist anakinra has been used in the treatment of certain autoinflammatory diseases.
  
  
  METHODS
  We describe two patients with severe acne and associated symptoms which led to the diagnosis PAPASH and PASH syndrome and who were treated with anakinra.
  
  
  RESULTS
  In the patient with PASH syndrome, inhibition of inflammation and almost complete healing of ulcers was observed. In the patient with PAPASH syndrome, partial response was achieved.
  
  
  CONCLUSIONS
  The therapeutic effect of anakinra in PASH syndrome and partly in PAPASH syndrome indicates an involvement of IL-1β in acne-associated autoinflammatory diseases.
authors.affiliation: Dermatologische Klinik, Universität Rom Tor Vergata, Rom, Italien.
authors.forename: S
authors.initials: S
authors.lastname: Faleri
authors.affiliation: Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland.
authors.forename: K
authors.initials: K
authors.lastname: Feichtner
authors.affiliation: Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland. thomas.ruzicka@med.uni-muenchen.de.
authors.forename: T
authors.initials: T
authors.lastname: Ruzicka
mesh_terms: D000152:Acne Vulgaris / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D000287:Administration, Topical; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D003879:Dermatologic Agents; D003937:Diagnosis, Differential; D056660:Hereditary Autoinflammatory Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage*; D008297:Male; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D003879:Dermatologic Agents; D053590:Interleukin 1 Receptor Antagonist Protein
keywords: Anakinra; Autoinflammation; Interleukin-1 receptor antagonist; Ulcer