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化脓性汗腺炎

Hidradenitis suppurativa.

作者信息

Sabat Robert, Alavi Afsaneh, Wolk Kerstin, Wortsman Ximena, McGrath Barry, Garg Amit, Szepietowski Jacek C

机构信息

Psoriasis Research and Treatment Center, Department of Dermatology, Venereology and Allergology and Institute of Medical Immunology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

出版信息

Lancet. 2025 Feb 1;405(10476):420-438. doi: 10.1016/S0140-6736(24)02475-9. Epub 2025 Jan 22.

Abstract

Hidradenitis suppurativa is a chronic inflammatory disease characterised by painful, deep-seated nodules, abscesses, and draining tunnels in the skin of axillary, inguinal, genitoanal, or inframammary areas. In recent years, the body of knowledge in hidradenitis suppurativa has advanced greatly. This disorder typically starts in the second or third decade of life. The average worldwide prevalence is 1% but varies geographically. Hidradenitis suppurativa has a profound negative effect on patients' quality of life and on the gross value added to society. Comorbidities (eg, metabolic syndrome, inflammatory arthritis, and inflammatory bowel disease) frequently accompany skin alterations, because of systemic inflammation. Pathogenesis of hidradenitis suppurativa is complex and includes innate immune mechanisms (eg, macrophages, neutrophils, IL-1β, tumour necrosis factor [TNF], and granulocyte colony-stimulating factor), T-cell mechanisms (eg, IL-17 and IFN-γ), and B-cell mechanisms (eg, associated with dermal tertiary lymphatic structures and autoantibodies). Chronic inflammation leads to irreversible skin damage with tunnel formation and morbid scarring. Current treatment includes drug therapy (for the initial, purely inflammatory phase), combined drug and surgical therapy (for the destructive phase), or surgery alone (for the burnout phase). The first systemic therapies approved for hidradenitis suppurativa targeting TNF (adalimumab) and IL-17 (secukinumab and bimekizumab) have expanded drug therapy options for moderate-to-severe disease, which were previously mainly restricted to oral antibiotics. Moreover, there is a robust pipeline of immunomodulatory drugs in various stages of development for hidradenitis suppurativa. Aims of management should include early intervention to prevent irreversible skin damage, adequate control of symptoms including pain, and mitigation of extra-cutaneous comorbidities, all requiring early diagnosis and an interdisciplinary, holistic and personalised approach.

摘要

化脓性汗腺炎是一种慢性炎症性疾病,其特征为腋窝、腹股沟、生殖器肛门或乳房下区域皮肤出现疼痛性深部结节、脓肿和引流道。近年来,化脓性汗腺炎的知识体系有了很大进展。这种疾病通常在人生的第二个或第三个十年开始。全球平均患病率为1%,但在不同地区有所差异。化脓性汗腺炎对患者的生活质量和社会总增加值有深远的负面影响。由于全身炎症,合并症(如代谢综合征、炎性关节炎和炎性肠病)经常伴随皮肤改变。化脓性汗腺炎的发病机制复杂,包括先天免疫机制(如巨噬细胞、中性粒细胞、白细胞介素-1β、肿瘤坏死因子[TNF]和粒细胞集落刺激因子)、T细胞机制(如白细胞介素-17和干扰素-γ)以及B细胞机制(如与皮肤三级淋巴结构和自身抗体相关)。慢性炎症导致不可逆的皮肤损伤,形成隧道和病态瘢痕。目前的治疗方法包括药物治疗(用于初始的单纯炎症阶段);药物与手术联合治疗(用于破坏性阶段);或单独手术(用于疾病消退阶段)。首批获批用于化脓性汗腺炎的全身治疗药物,靶向TNF的(阿达木单抗)和靶向白细胞介素-17的(司库奇尤单抗和比美吉珠单抗),为中重度疾病扩展了药物治疗选择,此前这些疾病主要局限于口服抗生素治疗。此外,有一系列处于不同研发阶段的用于化脓性汗腺炎的免疫调节药物。管理的目标应包括早期干预以防止不可逆的皮肤损伤、充分控制包括疼痛在内的症状以及减轻皮肤外合并症,所有这些都需要早期诊断以及跨学科、全面和个性化的方法。

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