Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany,
European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany,
Dermatology. 2021;237(5):673-697. doi: 10.1159/000509873. Epub 2020 Sep 17.
There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients.
A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission.
The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist's role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences.
越来越多的证据表明，（某些）化脓性汗腺炎合并症包括综合征，其中化脓性汗腺炎是主要的皮肤表现。这些明显的自身炎症性综合征及其诊断延迟可能对受影响的患者产生不利影响。
根据系统评价和荟萃分析的首选报告项目（PRISMA）指南，在 MEDLINE、EMBASE 和 CENTRAL 数据库上进行了系统评价。
检索到 64 篇关于综合征性化脓性汗腺炎的合格文章。确定的综合征包括已经描述的综合征（坏疽性脓皮病-痤疮-化脓性汗腺炎、化脓性关节炎-坏疽性脓皮病-化脓性汗腺炎、银屑病关节炎-坏疽性脓皮病-化脓性汗腺炎、坏疽性脓皮病-寻常痤疮-化脓性汗腺炎-强直性脊柱炎、滑膜炎-痤疮-脓疱病-骨质增生-骨炎）和进一步的新的症状组合。皮肤表现，包括化脓性汗腺炎病变，通常先于其他器官的表现。相当一部分患者的皮肤表现对常规治疗有抗药性，生物制剂的单一治疗不足以维持缓解。
随后将重点讨论检测到的综合征的发病机制和治疗结果。皮肤科医生在准确诊断和早期治疗管理化脓性汗腺炎方面具有关键作用。治疗的目的应该是有效预防或延迟自身炎症性疾病的发生及其不可逆转的后果。
