title: Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne.
abstract: Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)-1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence-based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.
authors.affiliation: Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark.
authors.forename: Caroline
authors.initials: C
authors.lastname: Vinkel
authors.affiliation: Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark.|Department of Biomedical Sciences, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Simon F
authors.initials: SF
authors.lastname: Thomsen
mesh_terms: D000152:Acne Vulgaris / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D001170:Arthritis, Infectious / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D015535:Arthritis, Psoriatic / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D003093:Colitis, Ulcerative / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D017497:Hidradenitis Suppurativa / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D017511:Pyoderma Gangrenosum / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D013577:Syndrome