Institute of Dermatology, F. Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.
Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Clin Dermatol. 2021 Mar-Apr;39(2):240-247. doi: 10.1016/j.clindermatol.2020.10.016. Epub 2020 Oct 16.
Hidradenitis suppurativa (HS) is a chronic inflammatory disease usually involving the major skin folds characterized by a multifactorial pathogenesis and a wide spectrum of clinical manifestations. It can also rarely present in association with other diseases as complex clinical syndromes, causing additional diagnostic and therapeutic challenges. Different etiopathologic factors contribute to follicular inflammation and suppurative lesions of syndromic HS, including follicular hyperkeratinization and plugging, as well as activation of autoinflammatory pathways. Patients with syndromic HS frequently have a severe disease course, presenting with atypical skin involvement, signs of systemic inflammation, and resistance to conventional treatments. Systematic classification of syndromic HS is based on clinical, pathogenetic, and genetic factors, but it is constantly evolving due to increased disease awareness. Treatment of syndromic HS is difficult and should be personalized on a case-by-case basis. Investigating syndromic HS can lead to useful insights on genetics and pathogenesis, translating into new clinical approaches for sporadic hidradenitis. We review the classification, clinical presentation, disease associations, and therapeutic management of syndromic HS, focusing mainly on its autoinflammatory syndromes PASH, PAPASH, PsAPASH, and PASS.
化脓性汗腺炎（HS）是一种慢性炎症性疾病，通常累及主要皮肤褶皱，具有多因素发病机制和广泛的临床表现。它也很少与其他疾病相关联，形成复杂的临床综合征，导致额外的诊断和治疗挑战。不同的病因病理因素导致综合征性 HS 的毛囊炎症和化脓性病变，包括毛囊过度角化和阻塞，以及自身炎症途径的激活。综合征性 HS 患者的疾病病程通常较为严重，表现为非典型皮肤受累、全身炎症迹象和对常规治疗的耐药性。综合征性 HS 的系统分类基于临床、发病机制和遗传因素，但由于疾病意识的提高，它在不断发展。综合征性 HS 的治疗较为困难，应根据具体情况进行个体化治疗。对综合征性 HS 的研究可以深入了解遗传和发病机制，为散发性化脓性汗腺炎提供新的临床治疗方法。我们主要关注其自身炎症性综合征 PASH、PAPASH、PsAPASH 和 PASS，综述了综合征性 HS 的分类、临床表现、疾病关联和治疗管理。
