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- Title
PAPASH, PsAPASH and PASS autoinflammatory syndromes: phenotypic heterogeneity, common biological signature and response to immunosuppressive regimens.
- Authors
Gottlieb, J.; Madrange, M.; Gardair, C.; Sbidian, E.; Frazier, A.; Wolkenstein, P.; Hickman, G.; Schneider, P.; Baudry, C.; Claudepierre, P.; Bertheau, P.; Richette, P.; Smahi, A.; Bachelez, H.
- Abstract
Dear Editor, Hidradenitis suppurativa (HS) (also known as suppurative hidradenitis) is a chronic inflammatory disease of skin appendages, commonly associating with other conditions including spondyloarthritis,[[1]] and is a major component of severe cutaneo-articular autoinflammatory syndromes (AIS), called PAPASH [Pyogenic Arthritis, Pyoderma gangrenosum, Acne, Suppurative Hidradenitis], PsAPASH (PSoriatic Arthritis, Pyoderma gangrenosum, Acne, Suppurative Hidradenitis) and PASS (Pyoderma gangrenosum, Acne, Suppurative hidradenitis and ankylosing Spondylitis).[[1], [3]] The clinical phenotypes of these AIS are ill-defined, and unlike PASH (Pyoderma gangrenosum, Acne, Suppurative Hidradenitis) or PAPA (Pyogenic Arthritis, Pyoderma gangrenosum, Acne) syndromes, still lack any biological or genetic marker. Moreover, their optimal therapeutic management remains unclear.[[4]] We report an in-depth description of the clinicopathological, radiological, immunobiological features and treatment outcomes in a series of nine patients diagnosed with PAPASH (n = 2), PsAPASH (n = 1) or PASS (n = 6), in three tertiary care centres between 2014 and 2016. Most patients had high CRP (mean: 68 mg L SP -1 sp ; normal < 5 mg L SP -1 sp ), which correlated with increased serum amyloid A levels in four of six patients analysed, and five showed polyclonal hypergammaglobulinaemia.
- Subjects
PSORIATIC arthritis; IRIDOCYCLITIS; HIDRADENITIS suppurativa; SYNDROMES
- Publication
British Journal of Dermatology, 2019, Vol 181, Issue 4, p866
- ISSN
0007-0963
- Publication type
Article
- DOI
10.1111/bjd.18003