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PAPASH, PsAPASH and PASS autoinflammatory syndromes: phenotypic heterogeneity, common biological signature and response to immunosuppressive regimens

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Hidradenitis suppurativa (HS) is a chronic inflammatory disease of skin appendages, commonly associating with other conditions including spondyloarthritis, and is a major component of severe cutaneo-articular autoinflammatory syndromes (AIS), called… Click to show full abstract

Hidradenitis suppurativa (HS) is a chronic inflammatory disease of skin appendages, commonly associating with other conditions including spondyloarthritis, and is a major component of severe cutaneo-articular autoinflammatory syndromes (AIS), called PAPASH (Pyogenic Arthritis, pyoderma gangrenosum/PG, Acne, HS), PsAPASH (Psoriatic Arthritis, PG, Acne, HS) and PASS (Ankylosing Spondylitis, Acne, PG and HS). The clinical phenotypes of these AIS are ill-defined and unlike PASH or PAPA syndromes, still lack any biological or genetic marker. Moreover, their optimal therapeutic management remains unclear. This article is protected by copyright. All rights reserved.

Keywords: autoinflammatory syndromes; papash psapash; pass autoinflammatory; autoinflammatory; psapash pass

Journal Title: British Journal of Dermatology
Year Published: 2019

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