Dias Philippe Raphael, Bolt Levin, Iking-Konert Christof, Arrigo Mattia, Huber Lars C
Department of Internal Medicine, Stadtspital Zurich Triemli, Zurich, Switzerland.
Division of Rheumatology, Stadtspital Zurich Triemli, Zurich, Switzerland.
Case Reports Immunol. 2024 Jan 25;2024:3671685. doi: 10.1155/2024/3671685. eCollection 2024.
Aseptic abscess syndrome (AAS) is a medical rarity. The combination of multiple abscess collections in different organs, negative microbiological studies, and the association with an inflammatory bowel disease is highly suggestive for an AAS. The AAS is an acute neutrophilic dermatosis, so "generalized pyoderma gangraenosum" or "generalized bullous sweet syndrome" might be used synonymously. It is important to note that the diagnosis of an AAS can be made only after careful exclusion of an infectious disease. Of interest, despite the severity of the inflammation, patients with AAS are commonly hemodynamically stable. To date, no studies have investigated the optimal regimen, dose, and duration of therapy. Corticosteroids are the cornerstone of immunosuppression during the acute phase. After the induction phase, therapy might be switched to anakinra or infliximab.
无菌性脓肿综合征(AAS)是一种医学上的罕见病症。不同器官出现多个脓肿、微生物学检查呈阴性以及与炎症性肠病相关联,这些情况高度提示为AAS。AAS是一种急性嗜中性皮病,因此“全身性坏疽性脓皮病”或“全身性大疱性Sweet综合征”可能是同义词。需要注意的是,只有在仔细排除传染病后才能做出AAS的诊断。有趣的是,尽管炎症严重,但AAS患者通常血流动力学稳定。迄今为止,尚无研究调查最佳治疗方案、剂量和疗程。在急性期,皮质类固醇是免疫抑制的基石。诱导期过后,治疗可能会改用阿那白滞素或英夫利昔单抗。