Faleri S, Feichtner K, Ruzicka T
Dermatologische Klinik, Universität Rom Tor Vergata, Rom, Italien.
Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland.
Hautarzt. 2016 Nov;67(11):897-901. doi: 10.1007/s00105-016-3840-0.
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.
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.
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.
The therapeutic effect of anakinra in PASH syndrome and partly in PAPASH syndrome indicates an involvement of IL-1β in acne-associated autoinflammatory diseases.
近年来，痤疮被描述为自身炎症性疾病的一种症状，如PASH（坏疽性脓皮病 - PG、痤疮和化脓性汗腺炎 - SH）和PAPASH（PG、痤疮、化脓性关节炎和SH）。自身炎症性疾病的发病机制尚未完全明确；然而，基于白细胞介素-1β（IL-1β）可能的参与，重组人白细胞介素-1受体拮抗剂阿那白滞素已被用于治疗某些自身炎症性疾病。
我们描述了两名患有重度痤疮及相关症状的患者，他们被诊断为PAPASH和PASH综合征，并接受了阿那白滞素治疗。
在患有PASH综合征的患者中，观察到炎症得到抑制且溃疡几乎完全愈合。在患有PAPASH综合征的患者中，取得了部分疗效。
阿那白滞素在PASH综合征以及部分PAPASH综合征中的治疗效果表明IL-1β参与了痤疮相关的自身炎症性疾病。
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