PASS Syndrome: An IL-1-Driven Autoinflammatory Disease.
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 spond...
| Publicado en: | Dermatology (10188665) Vol. 232; no. 2; pp. 254 - 259 |
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| Autores principales: | , , , , , , , , |
| Formato: | case study Journal Article |
| Publicado: |
Karger AG
Apr2016
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=115006882&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 115006882 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 10188665 NIG jtl: Dermatology (10188665) issn: 10188665 maglogo: N pubinfo: dt: Apr2016 vid: 232 iid: 2 pid: 2485 pub: Karger AG artinfo: ui: 115006882 115006882 NLM26919742 115006882 10.1159/000443648 NLM26919742 115006882 ppf: 254 ppct: 5 formats: tig: atl: PASS Syndrome: An IL-1-Driven Autoinflammatory Disease. aug: au: Leuenberger, Mathieu Berner, Jeanne Di Lucca, Julie Fischer, Lara Kaparos, Nikolaos Conrad, Curdin Hohl, Daniel So, alexander Gilliet, Michel affil: Dermatology sug: subj: Autoimmune Diseases Drug Therapy Antirheumatic Agents Therapeutic Use Acne Vulgaris Diagnosis Proteins Therapeutic Use Spondylitis, Ankylosing Diagnosis Hidradenitis Suppurativa Diagnosis Pyoderma Gangrenosum Diagnosis Autoimmune Diseases Diagnosis Male Autoimmune Diseases Blood Syndrome Interleukin 1 Blood Adult Psychological Tests Adult: 19-44 years Male ab: 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. pubtype: Academic Journal doctype: case study Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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