Division of Dermatology and Venereology, Department of Medicine Solna and Center for Molecular Medicine, Karolinska Institutet, 17176 Stockholm, Sweden.
Department of Clinical Medicine and Surgery, University of Naples Federico II, 80131 Naples, Italy.
Cells. 2021 Aug 15;10(8):2094. doi: 10.3390/cells10082094.
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease primarily affecting apocrine gland-rich areas of the body. It is a multifactorial disease in which genetic and environmental factors play a key role. The primary defect in HS pathophysiology involves follicular occlusion of the folliculopilosebaceous unit, followed by follicular rupture and immune responses. Innate pro-inflammatory cytokines (e.g., IL-1β, and TNF-α); mediators of activated T helper (Th)1 and Th17 cells (e.g., IFN-γ, and IL-17); and effector mechanisms of neutrophilic granulocytes, macrophages, and plasma cells are involved. On the other hand, HS lesions contain anti-inflammatory mediators (e.g., IL-10) and show limited activity of Th22 cells. The inflammatory vicious circle finally results in pain, purulence, tissue destruction, and scarring. HS pathogenesis is still enigmatic, and a valid animal model for HS is currently not available. All these aspects represent a challenge for the development of therapeutic approaches, which are urgently needed for this debilitating disease. Available treatments are limited, mostly off-label, and surgical interventions are often required to achieve remission. In this paper, we provide an overview of the current knowledge surrounding HS, including the diagnosis, pathogenesis, treatments, and existing translational studies.
化脓性汗腺炎(HS)是一种主要影响顶泌汗腺丰富区域的慢性炎症性皮肤病。它是一种多因素疾病,遗传和环境因素起着关键作用。HS 病理生理学的主要缺陷涉及毛囊皮脂腺单位的滤泡阻塞,随后是滤泡破裂和免疫反应。先天促炎细胞因子(例如,IL-1β和 TNF-α);激活的辅助性 T 细胞(Th)1 和 Th17 细胞的介质(例如,IFN-γ和 IL-17);以及中性粒细胞、巨噬细胞和浆细胞的效应机制均参与其中。另一方面,HS 病变含有抗炎介质(例如,IL-10),并且 Th22 细胞的活性有限。炎症的恶性循环最终导致疼痛、化脓、组织破坏和瘢痕形成。HS 的发病机制仍然神秘莫测,目前尚无有效的 HS 动物模型。所有这些方面都对治疗方法的发展构成了挑战,而这种使人衰弱的疾病迫切需要新的治疗方法。现有的治疗方法有限,大多是未经批准的,通常需要手术干预才能达到缓解。本文综述了目前关于 HS 的知识,包括诊断、发病机制、治疗方法和现有的转化研究。