PDF(Pubmed)
Abstract：
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare condition characterized by immune complex-mediated urticarial lesions with histological features of leukocytoclastic vasculitis, low serum complement levels, and is frequently associated with systemic manifestations. Its pathophysiology is poorly understood. We present a patient who presented with abdominal pain and skin rash. Extensive work-up was performed including skin biopsy, and the presence of angioedema, oral ulcers, low complement level, leukocytic vasculitis, and persistent eosinophilia ultimately led to the diagnosis of HUVS. This case highlights the importance of recognizing and differentiating HUVS from other cutaneous diseases, which in turn helps to optimally manage these patients.
摘要：
低补体血症性荨麻疹性血管炎综合征（HUVS）是一种罕见的疾病，其特征是免疫复合物介导的荨麻疹病变，具有白细胞碎裂性血管炎的组织学特征，低血清补体水平，并经常与系统表现有关。对其病理生理学了解甚少。我们介绍了一名出现腹痛和皮疹的患者。进行了广泛的检查，包括皮肤活检，血管性水肿的存在，口腔溃疡,补体水平低,白细胞性血管炎,持续的嗜酸性粒细胞增多最终导致HUVS的诊断。该病例强调了识别和区分HUVS与其他皮肤疾病的重要性，这反过来又有助于优化管理这些患者。
