Iannello S, Asmundo G O, Cataliotti A, Floridia S, Loreno M, Trovato G, Vintaloro G, Belfiore F
Istituto di Medicina Interna e Specialità Internistiche, Ospedale Garibaldi, Catania.
Minerva Med. 1997 Nov;88(11):459-67.
Urticarial vasculitis (UV) is a primary syndrome or a cutaneous vasculitic lesion occurring in the course of a collagen disease, as the systemic lupus erythematosus (LE). UV is a recently recognized disorder which affects most exclusively the female sex and may be differentiated from common (nonvasculitic) urticaria because it is characterized by inflammation and necrosis of blood vessels (vasculitis). UV and common urticaria may be induced by a variety of factors and pathogenetic mechanisms. It seems that a continuum exists, ranging from benign cutaneous lesions of urticaria to vasculitis with strong immunological involvement. On the basis of the clinical evaluation, two major groups of UV have been classified, the normocomplementemic, with a less severe clinical course, and the hypocomplementemic UV, a rare immune complex-mediated disorder related to LE as for as the similar pathogenesis and systemic involvement are concerned. A case of a young female patient with a unique syndrome characterized by crises of urticarial recurrent painful lesions of unknown origin, associated with angioedema of the tongue and soft palate, severe malaise, arthralgias and abdominal pain is reported. Dyspnea and cough were sometimes present, but fever was absent. Symptoms were caused by physical stress, heat or pressure and were little responsive to corticosteroids. Although skin biopsy did not prove with certainty the occurrence of vasculitis, clinical data and laboratory findings (normal levels of complement fractions, raised erythrocyte sedimentation rate, presence of cryoglobulins and ASMA, nDNA and AMA autoantibodies and absence of ANA autoantibodies and LE cells) suggest a normocomplementemic UV, excluding a classic LE. The family doctors and the internist must become familiar with the "lupus-like" syndromes, which include UV. In fact, a correct diagnosis of this syndrome is important because, although prognosis of UV may not be severe, the possibility exists of a systemic involvement (mainly renal) with progression to LE.
荨麻疹性血管炎（UV）是一种原发性综合征或发生于胶原病（如系统性红斑狼疮，LE）病程中的皮肤血管性病变。UV是一种最近才被认识的疾病，主要影响女性，并且可与普通（非血管性）荨麻疹相鉴别，因为其特征是血管炎症和坏死（血管炎）。UV和普通荨麻疹可能由多种因素和发病机制诱发。似乎存在一个连续谱，从荨麻疹的良性皮肤病变到有强烈免疫参与的血管炎。根据临床评估，UV已被分为两大类，即补体正常的UV，其临床病程较轻；以及补体低下的UV，这是一种罕见的免疫复合物介导的疾病，就发病机制和全身受累情况而言与LE相关。本文报告了一例年轻女性患者，其具有独特的综合征，特征为不明原因的荨麻疹反复发作性疼痛性皮损，伴有舌部和软腭血管性水肿、严重不适、关节痛和腹痛。有时会出现呼吸困难和咳嗽，但无发热。症状由身体应激、热或压力诱发，对皮质类固醇反应不佳。尽管皮肤活检未能确切证实血管炎的发生，但临床资料和实验室检查结果（补体成分水平正常、红细胞沉降率升高、存在冷球蛋白以及抗平滑肌抗体、抗双链DNA抗体和抗线粒体抗体，且无抗核抗体和狼疮细胞）提示为补体正常的UV，可排除典型的LE。家庭医生和内科医生必须熟悉包括UV在内的“狼疮样”综合征。事实上，正确诊断该综合征很重要，因为尽管UV的预后可能不严重，但仍有可能发生全身受累（主要是肾脏）并进展为LE。
