Schäkel K, Meurer M
Klinik und Poliklinik für Dermatologie am Universitätsklinikum Carl Gustav Carus der Technischen Universität Dresden.
Hautarzt. 2008 May;59(5):374-81. doi: 10.1007/s00105-008-1546-7.
Vasculitis is characterized by an inflammatory reaction of vessel walls with damage to the dependent tissues. Forms of vasculitis which frequently have skin changes include leukocytoclastic angiitis (LcV), Henoch-Schönlein purpura (HSP), cutaneous polyarteriitis nodosum (cPAN), erythema elevatum et diutinum (EED) and urticarial vasculitis (UV). In other forms of vasculitis, systemic manifestations predominate but there are a variety of skin changes. Kawasaki disease (MK), cryoglobulinemic vasculitis (kV), Wegener granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangitis (MPA) belong to this group. The causes of vasculitis are heterogeneous. Triggers include infections, drugs, collagen vascular diseases, autoimmune diseases and lymphoproliferative disorders. Idiopathic vasculitis, particularly LcV and EED, occur only once and have a self-limited course. The diagnostic work up depends on the clinical picture and includes inflammatory markers, circulating immune complexes, different types of cryoglobulins and anti-neutrophilic cytoplasmic antibodies, collagen vascular disease specific autoantibodies and additional hematological studies. Vasculitis can manifest in many organs and requires a thorough work up specifically in cases where WG, MPA, CSS and PAN are under consideration.
血管炎的特征是血管壁发生炎症反应并损害相关组织。常伴有皮肤改变的血管炎类型包括白细胞破碎性血管炎(LcV)、过敏性紫癜(HSP)、皮肤型结节性多动脉炎(cPAN)、持久性隆起性红斑(EED)和荨麻疹性血管炎(UV)。在其他血管炎类型中,以全身表现为主,但也有多种皮肤改变。川崎病(MK)、冷球蛋白血症性血管炎(kV)、韦格纳肉芽肿(WG)、变应性肉芽肿性血管炎(CSS)和显微镜下多血管炎(MPA)属于这一组。血管炎的病因多种多样。触发因素包括感染、药物、胶原血管病、自身免疫性疾病和淋巴增殖性疾病。特发性血管炎,尤其是LcV和EED,仅发作一次且病程自限。诊断检查取决于临床表现,包括炎症标志物、循环免疫复合物、不同类型的冷球蛋白和抗中性粒细胞胞浆抗体、胶原血管病特异性自身抗体以及其他血液学检查。血管炎可累及多个器官,在考虑为WG、MPA、CSS和PAN的情况下尤其需要进行全面检查。