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, et al.
The British journal of dermatology. Date of publication 2019 Apr 13;volume ():.
1. Br J Dermatol. 2019 Apr 13. doi: 10.1111/bjd.18003. [Epub ahead of print]
PAPASH, PsAPASH and PASS autoinflammatory syndromes: Phenotypic heterogeneity,
common biological signature and response to immunosuppressive regimens.
Gottlieb J(1), Madrange M(2), Gardair C(3)(4), Sbidian E(5)(6)(7), Frazier A(8),
Wolkenstein P(5)(6)(7), Hickman G(1), Schneider P(1), Baudry C(4)(9),
Claudepierre P(6)(7)(10), Bertheau P(3)(4), Richette P(8)(11), Smahi A(2),
Bachelez H(1)(2)(4).
Author information:
(1)Department of Dermatology, APHP Hôpital St-Louis, Paris.
(2)UMR Inserm 1163, Laboratoire de Génétique des Maladies Cutanées, Institut
Imagine, Necker Hospital, Paris.
(3)Department of Pathology, APHP Hôpital St-Louis, Paris.
(4)Sorbonne Paris Cité Université Paris Diderot, Paris.
(5)Department of Dermatology, APHP Hôpital Henri Mondor, Créteil.
(6)Université Paris Est Créteil, Créteil, France.
(7)EA 7379 - EpidermE, Créteil, France.
(8)Department of Rheumatology, APHP Hôpital Lariboisière, Paris.
(9)Department of Gastro-enterology, APHP Hôpital St-Louis, Paris.
(10)Department of Rheumatology, APHP Hôpital Henri Mondor, Créteil.
(11)Inserm U1132, UFR de médecine, Paris Diderot University, Paris, France.
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.
This article is protected by copyright. All rights reserved.
DOI: 10.1111/bjd.18003
PMID: 30980721
