PDF(Pubmed)
Abstract：
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterized by chronic urticaria, systemic vasculitis, and hypocomplementemia, posing significant diagnostic challenges due to its overlap with common conditions and varied systemic manifestations. We report the case of a 36-year-old female with a history of post-birth cerebral hemorrhage and seizure disorder, who presented with abdominal pain, diarrhea, and subtle urticarial lesions. Initial investigations by gastroenterology suggested inflammatory bowel disease (IBD), but persistent symptoms and evolving cutaneous signs prompted further evaluation. A skin biopsy demonstrated leukocytoclastic vasculitis, while serological tests showed hypocomplementemia and positive antineutrophil cytoplasmic antibodies (ANCA), confirming HUVS. The patient\'s management included high-dose corticosteroids and mycophenolate mofetil, with partial symptom relief. Subsequent introduction of rituximab markedly improved her gastrointestinal and dermatological symptoms, highlighting its effectiveness in treating refractory HUVS. This case emphasizes the necessity for vigilance, interdisciplinary collaboration, and personalized treatment adaptations in managing HUVS.
摘要：
低补体血症性荨麻疹性血管炎综合征（HUVS）是一种罕见的自身免疫性疾病，以慢性荨麻疹为特征，全身性血管炎,和低补体血症，由于其与常见疾病和不同的全身表现重叠，因此构成了重大的诊断挑战。我们报告了一例36岁的女性，有出生后脑出血和癫痫发作史，出现腹痛的人，腹泻,和微妙的荨麻疹病变。胃肠病学的初步调查显示炎症性肠病（IBD），但是持续的症状和不断发展的皮肤体征促使进一步评估。皮肤活检显示白细胞碎裂性血管炎，而血清学检查显示低补体血症和抗中性粒细胞胞浆抗体（ANCA）阳性，确认HUVS。患者的治疗包括大剂量皮质类固醇和霉酚酸酯，部分症状缓解。随后利妥昔单抗的引入显着改善了她的胃肠道和皮肤病学症状，强调其治疗难治性HUVS的有效性。这个案例强调了警惕的必要性,跨学科合作,和个性化治疗适应管理HUVS。
