Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark.
Department of Biomedical Sciences, University of Copenhagen, Copenhagen, Denmark.
Int J Dermatol. 2017 Aug;56(8):811-818. doi: 10.1111/ijd.13603. Epub 2017 Mar 27.
Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)-1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence-based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.
与化脓性汗腺炎（HS）和/或痤疮相关的自身炎症性综合征较为罕见，但如果诊断和治疗不当，可能会导致严重的身体残疾。这些疾病具有共同的发病机制，涉及失调的固有免疫系统，白细胞介素（IL）-1 信号异常，导致无菌性中性粒细胞炎症。临床特征为反复发作的发热、疼痛性关节炎和符合 HS、痤疮和坏疽性脓皮病（PG）的皮肤病变，同时伴有血液中系统性炎症标志物升高。迄今为止，文献中已经报道了几种具有临床差异的综合征，包括坏疽性脓皮病、痤疮和化脓性关节炎（PAPA）、坏疽性脓皮病、痤疮和化脓性汗腺炎（PASH）、坏疽性脓皮病、痤疮和脊椎关节炎（PASS）、坏疽性脓皮病、痤疮、化脓性关节炎和化脓性汗腺炎（PAPASH）、银屑病关节炎、坏疽性脓皮病、痤疮和化脓性汗腺炎（PsAPASH）和坏疽性脓皮病、痤疮和溃疡性结肠炎（PAC）。这些综合征的罕见性使得基于证据的治疗指南的建立变得复杂。此外，由于对标准治疗方法缺乏反应，治疗可能具有挑战性。因此，提高对这些疾病的认识很重要，以便优化疾病管理，最终提高患者的生活质量。
