Hansen-Abeck Inga, Rünger Alessandra, Piepke Lisa, Kött Julian, Giordano-Rosenbaum Anna, Menz Anne, Abeck Finn, Schneider Stefan W
Department of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Institute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
J Dermatol. 2025 Jan;52(1):43-49. doi: 10.1111/1346-8138.17545. Epub 2024 Nov 18.
Non-IgA vasculitis is a rare disease that belongs to the group of small-vessel vasculitides. Due to nomenclature and classification changes introduced in 2018, there are few published data under this name. The aim of this study is to characterize non-IgA vasculitis as an independent vasculitis entity in terms of demographic, clinical, and treatment-related features. A retrospective data analysis of patients with biopsy-confirmed non-IgA vasculitis treated at the Department of Dermatology at the University Medical Center Hamburg-Eppendorf between January 1, 2018, and December 31, 2022, was performed. A total of 28 patients with non-IgA vasculitis were included; 53.6% (15/28) were women and 42.9% (12/28) were older than 71 years. Previous infection as a possible triggering factor was found in 42.6% (12/28) of the cases. Palpable purpura was the most common skin finding (78.6%, 22/28) and 28.6% patients (8/28) had skin lesions above the waist. On direct immunofluorescence, C3 (89.3%, 25/28) was the most frequent deposition, followed by fibrinogen (71.4%, 20/28) and IgM (53.6%, 15/28). Hospitalization was required in 85.7% (24/28), with a mean hospital stay of 9.4 ± 4.1 days. No fatal courses were reported. This study is the first characterization of non-IgA vasculitis based on patient cases from Germany and contributes to a better understanding of non-IgA vasculitis as an independent entity. Non-IgA vasculitis primarily affects older patients of both sexes, with most cases having an identifiable trigger. Our results indicate that cutaneous manifestations often extend beyond the lower legs. Treatment is usually required in the inpatient setting and requires a longer stay than other dermatological conditions. With proper treatment, the disease is not expected to be fatal.
非IgA血管炎是一种罕见疾病，属于小血管血管炎范畴。由于2018年引入了命名和分类变化，以该名称发表的数据很少。本研究的目的是从人口统计学、临床和治疗相关特征方面，将非IgA血管炎表征为一种独立的血管炎实体。对2018年1月1日至2022年12月31日在汉堡-埃彭多夫大学医学中心皮肤科接受活检确诊的非IgA血管炎患者进行了回顾性数据分析。共纳入28例非IgA血管炎患者；53.6%（15/28）为女性，42.9%（12/28）年龄超过71岁。42.6%（12/28）的病例中发现既往感染是可能的触发因素。可触及性紫癜是最常见的皮肤表现（78.6%，22/28），28.6%的患者（8/28）腰部以上有皮肤病变。直接免疫荧光检查显示，C3（89.3%，25/28）是最常见的沉积物，其次是纤维蛋白原（71.4%，20/28）和IgM（53.6%，15/28）。85.7%（24/28）的患者需要住院治疗，平均住院时间为9.4±4.1天。未报告致命病程。本研究是基于德国患者病例对非IgA血管炎的首次表征，有助于更好地理解非IgA血管炎作为一种独立实体。非IgA血管炎主要影响老年男女患者，大多数病例有可识别的触发因素。我们的结果表明，皮肤表现通常不仅限于小腿。治疗通常需要住院，且住院时间比其他皮肤病长。经过适当治疗，该疾病预计不会致命。
