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病例报告:PsAPSASH 综合征:一种综合征性化脓性汗腺炎的替代表型,用 IL-17A 抑制剂司库奇尤单抗治疗。

Case Report: PsAPSASH syndrome: an alternative phenotype of syndromic hidradenitis suppurativa treated with the IL-17A inhibitor secukinumab.

机构信息

Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg, Dessau, 06847, Germany.

European Hidradenitis Suppurativa Foundation e.V., Dessau, 06847, Germany.

出版信息

F1000Res. 2021 May 13;10:381. doi: 10.12688/f1000research.52100.2. eCollection 2021.

Abstract

Syndromic hidradenitis suppurativa (HS) is a form of symptom constellations, which differs from the familial and genetic form and comprises predominantly osteoarticular manifestations. Many forms include pyoderma gangrenosum and acne (PASH), pyogenic arthritis (PAPASH), spondyloarthritis (PASS) and psoriatic arthritis (PsAPASH) and are categorized in the autoinflammatory syndromes. Anti-TNF-α and anti-IL-1a blockade are between the therapeutic approaches that improve skin symptoms and prevent permanent osteoarticular damage. This case report refers to the successful treatment of a mixed phenotype of the aforementioned symptoms using the IL-17A inhibitor secukinumab after initial treatment with adalimumab. The therapy improved both cutaneous and reported osteoarticular symptoms. Different approaches for these recalcitrant HS syndromes are essential in order to achieve long-term remission for those patients.

摘要

化脓性汗腺炎(HS)综合征是一种症状组合形式,与家族性和遗传性形式不同,主要包括骨关节炎表现。许多形式包括坏疽性脓皮病和痤疮(PASH)、化脓性关节炎(PAPASH)、脊柱关节炎(PASS)和银屑病关节炎(PsAPASH),并归类于自身炎症性综合征。抗 TNF-α 和抗 IL-1a 阻断是改善皮肤症状和预防永久性骨关节炎损伤的治疗方法之一。本病例报告介绍了一例混合表型的上述症状患者,在初始阿达木单抗治疗后,使用 IL-17A 抑制剂司库奇尤单抗成功治疗。该疗法改善了皮肤和报告的骨关节炎症状。对于这些难治性 HS 综合征,需要采用不同的方法,以实现这些患者的长期缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f55/8424493/3c286c9132b7/f1000research-10-57760-g0000.jpg

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