PASS Syndrome: An IL-1-Driven Autoinflammatory Disease


Journal: Dermatology (Basel) 2016 Feb;

Author(s): Leuenberger M, Berner J, Di Lucca J, Fischer L, Kaparos N, Conrad C, Hohl D, So A, Gilliet M

PASS syndrome is a rare inflammatory disease characterized by a chronic-relapsing course of pyoderma gangrenosum, acne vulgaris, hidradenitis suppurativa and ankylosing spondylitis. Here, we describe a case of a patient with spontaneously recurrent purulent skin lesions along with seronegative spondylarthritis consistent with the PASS syndrome. During his disease exacerbation, the patient displayed episodes of fever along with elevated serum levels of interleukin (IL)-1β. Skin lesions were characterized by sterile neutrophilic infiltrates and showed a rapid response to the IL-1 receptor antagonist anakinra (Kineret®) consistent with the autoinflammatory nature of this disease. However, unlike other autoinflammatory diseases such as PAPA and PAPASH, we did not find mutations in the gene PSTPIP1, raising the possibility that other specific mutations in the IL-1 pathway may be involved.

Dermatology Journal and/or Publisher

Journal Name: Dermatology (Basel, Switzerland)
Journal Abbreviation: Dermatology (Basel)
Journal Volume: , Issue:
Journal Date Published: 2016-02-27

National Center for Biotechnology Information

PMI ID: 26919742
Created: 2016-02-26

Abstract Source: National Center for Biotechnology Information, U.S. National Library of Medicine Abstract Query for “Hidradenitis suppurativa”


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