PASS Syndrome: An IL-1-Driven Autoinflammatory Disease
Autor: | L. Fischer, Michel Gilliet, So A, Di Lucca J, M. Leuenberger, Kaparos N, Berner J, Daniel Hohl, Curdin Conrad |
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Rok vydání: | 2015 |
Předmět: |
Adult
Male Interleukin-1beta Dermatology Disease Autoimmune Diseases 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Acne Vulgaris medicine Humans Hidradenitis suppurativa Spondylitis Ankylosing Spondylitis Acne 030203 arthritis & rheumatology Anakinra Ankylosing spondylitis business.industry Interleukin Syndrome medicine.disease Pyoderma Gangrenosum Hidradenitis Suppurativa Interleukin 1 Receptor Antagonist Protein Antirheumatic Agents Immunology business Pyoderma gangrenosum medicine.drug |
Zdroj: | Dermatology (Basel, Switzerland). 232(2) |
ISSN: | 1421-9832 |
Popis: | 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. |
Databáze: | OpenAIRE |
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