S Faleri1, K Feichtner2, T Ruzicka3. 1. Dermatologische Klinik, Universität Rom Tor Vergata, Rom, Italien. 2. Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland. 3. Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland. thomas.ruzicka@med.uni-muenchen.de.
Abstract
BACKGROUND: In recent years, acne has been described as a symptom of autoinflammatory diseases, such as PASH (pyoderma gangrenosum - PG, acne and suppurative hidradenitis - SH) and PAPASH (PG, acne, pyogenic arthritis, and SH). The pathogenesis of autoinflammatory diseases is not fully understood; however, based on the possible involvement of IL-1β, the recombinant human interleukin-1 receptor antagonist anakinra has been used in the treatment of certain autoinflammatory diseases. METHODS: We describe two patients with severe acne and associated symptoms which led to the diagnosis PAPASH and PASH syndrome and who were treated with anakinra. RESULTS: In the patient with PASH syndrome, inhibition of inflammation and almost complete healing of ulcers was observed. In the patient with PAPASH syndrome, partial response was achieved. CONCLUSION: The therapeutic effect of anakinra in PASH syndrome and partly in PAPASH syndrome indicates an involvement of IL-1β in acne-associated autoinflammatory diseases.
BACKGROUND: In recent years, acne has been described as a symptom of autoinflammatory diseases, such as PASH (pyoderma gangrenosum - PG, acne and suppurative hidradenitis - SH) and PAPASH (PG, acne, pyogenic arthritis, and SH). The pathogenesis of autoinflammatory diseases is not fully understood; however, based on the possible involvement of IL-1β, the recombinant human interleukin-1 receptor antagonist anakinra has been used in the treatment of certain autoinflammatory diseases. METHODS: We describe two patients with severe acne and associated symptoms which led to the diagnosis PAPASH and PASH syndrome and who were treated with anakinra. RESULTS: In the patient with PASH syndrome, inhibition of inflammation and almost complete healing of ulcers was observed. In the patient with PAPASH syndrome, partial response was achieved. CONCLUSION: The therapeutic effect of anakinra in PASH syndrome and partly in PAPASH syndrome indicates an involvement of IL-1β in acne-associated autoinflammatory diseases.
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