Dayanand Lekhana, A Y Rahil, Nk Rohan Krishna
Internal Medicine, Fortis Hospital, Rajajinagar, Bengaluru, IND.
Cureus. 2025 Jan 1;17(1):e76723. doi: 10.7759/cureus.76723. eCollection 2025 Jan.
Adult-onset Still's disease (AOSD) is an uncommon clinical condition with an uncertain cause, characterized by arthritis, fever, evanescent rash, and other systemic presentations. This case report describes a 26-year-old female who had a fever, arthralgia, vomiting, sore throat, bilateral distal extremities edema, hypertension, and normal ferritin. She was diagnosed with AOSD using the Yamaguchi criteria based on exclusion and was treated with hydroxychloroquine and sulfasalazine. The patient's arthralgia improved significantly. However, she returned with complaints of a diffuse erythematous burning, pruritic, maculopapular, non-evanescent rash caused by a severe reaction to sulfasalazine, with a skin biopsy revealing urticarial vasculitis, with probable DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome. This was treated with topical and short-course oral steroids, intravenous antibiotics, and Janus kinase (JAK) inhibitor, improving her condition remarkably. To the best of our knowledge, there are no prior reports of a case of AOSD with normal ferritin levels that also exhibited a severe reaction to sulfasalazine, compounded by complications due to NSAIDs (nonsteroidal anti-inflammatory drugs) and steroid use. This rarity distinguishes our case report.
成人斯蒂尔病（AOSD）是一种病因不明的罕见临床病症，其特征为关节炎、发热、一过性皮疹及其他全身表现。本病例报告描述了一名26岁女性，她出现发热、关节痛、呕吐、咽痛、双侧远端肢体水肿、高血压且铁蛋白水平正常。根据排除标准，她依据山口标准被诊断为AOSD，并接受了羟氯喹和柳氮磺胺吡啶治疗。患者的关节痛明显改善。然而，她因对柳氮磺胺吡啶严重过敏反应而再次就诊，主诉有弥漫性红斑、烧灼感、瘙痒、斑丘疹且非一过性皮疹，皮肤活检显示为荨麻疹性血管炎，可能伴有药物超敏反应伴嗜酸性粒细胞增多和全身症状（DRESS）综合征。对此采用了外用和短疗程口服类固醇、静脉用抗生素及Janus激酶（JAK）抑制剂治疗，使她的病情显著改善。据我们所知，此前尚无铁蛋白水平正常的AOSD病例同时对柳氮磺胺吡啶出现严重反应并合并非甾体抗炎药（NSAIDs）和类固醇使用所致并发症的报道。这种罕见性使我们的病例报告具有独特性。
