Estrada Rodríguez J L, López Serrano C, Belchi Hernández J, Florido López F, Martínez Gómez W
Servicio de Alergia e Inmunología Clínica, Policlínica de Adultos, Hospital La Paz, Madrid, Spain.
J Investig Allergol Clin Immunol. 1991 Feb;1(1):69-71.
A patient with hypocomplementemic urticarial vasculitic syndrome (HUV) is presented. This is an immunological pathology, limited to skin or multisystemic, that requires a differential diagnosis with erythematosus systemic lupus on the same occasions. The ever-present symptom is skin participation, such as urticaria-angioedema or fixed exanthema; biopsy shows necrotizing venulitis with polymorphonuclear infiltration and leukocytoclastic powder. Typical laboratory data are: diminished C3, C4 and C1q; C1 inhibition can be low or normal; the more characteristic finding is the presence of C1q associated immunocomplexes. Leukocytoclastic necrotizing vasculitis was found in the skin biopsy. During the course of illness (three years) the patient presented moderate cutaneous symptoms and asthma, without other systemic participation. During this period, antihistamines and, occasionally, corticoids were administered with improvement. Moreover, the patient presented urticaria related to ampicillin ingestion, and furthermore, the presence of anaphylaxis to beta-lactam was diagnosed in vivo and specific IgE was found in the laboratory study. This feature was previously observed by other authors; however, we cannot determine why the IgE-mediated allergy to beta-lactam and a complement pathology like HUV are related.
本文报告了一例低补体血症性荨麻疹性血管炎综合征（HUV）患者。这是一种免疫病理学疾病，可局限于皮肤或累及多系统，在某些情况下需要与系统性红斑狼疮进行鉴别诊断。常见症状是皮肤受累，如荨麻疹 - 血管性水肿或固定性皮疹；活检显示坏死性小静脉炎伴多形核细胞浸润和白细胞破碎性碎屑。典型的实验室检查数据包括：C3、C4和C1q降低；C1抑制因子可降低或正常；更具特征性的发现是存在与C1q相关的免疫复合物。皮肤活检发现白细胞破碎性坏死性血管炎。在病程（三年）中，患者出现中度皮肤症状和哮喘，无其他系统受累。在此期间，给予抗组胺药，偶尔给予皮质类固醇，症状有所改善。此外，患者出现与服用氨苄西林相关的荨麻疹，并且在体内诊断出对β-内酰胺类药物过敏，实验室研究发现了特异性IgE。其他作者之前也观察到了这一特征；然而，我们无法确定为什么IgE介导的对β-内酰胺类药物的过敏与像HUV这样的补体病理学有关。
