title: PASS Syndrome: An IL-1-Driven Autoinflammatory Disease.
abstract: 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.
authors.affiliation: Department of Dermatology, University Hospital Center CHUV, Lausanne, Switzerland.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Leuenberger
authors.forename: Jeanne
authors.initials: J
authors.lastname: Berner
authors.forename: Julie
authors.initials: J
authors.lastname: Di Lucca
authors.forename: Lara
authors.initials: L
authors.lastname: Fischer
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Kaparos
authors.forename: Curdin
authors.initials: C
authors.lastname: Conrad
authors.forename: Daniel
authors.initials: D
authors.lastname: Hohl
authors.forename: Alexander
authors.initials: A
authors.lastname: So
authors.forename: Michel
authors.initials: M
authors.lastname: Gilliet
mesh_terms: D000152:Acne Vulgaris / Q000175:diagnosis*; D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy*; D017497:Hidradenitis Suppurativa / Q000175:diagnosis*; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D053583:Interleukin-1beta / Q000097:blood; D008297:Male; D017511:Pyoderma Gangrenosum / Q000175:diagnosis*; D013167:Spondylitis, Ankylosing / Q000175:diagnosis*; D013577:Syndrome
chemical_list: D018501:Antirheumatic Agents; C508585:IL1B protein, human; D053590:Interleukin 1 Receptor Antagonist Protein; D053583:Interleukin-1beta