Sunderkötter Cord, Michl Christiane
Universitätsklinikum Halle (Saale), Universitätsklinik und Poliklinik für Dermatologie und Venerologie, Martin-Luther-Universität Halle-Wittenberg, Ernst-Grube-Str. 40, 06120, Halle (Saale), Deutschland.
Internist (Berl). 2019 Aug;60(8):805-813. doi: 10.1007/s00108-019-0642-3.
Cutaneous vasculitides present with typical clinical signs depending on the size and distribution of the affected vessels. Since there are no large vessels in the skin, giant cell arteritis and Takayasu's arteritis only rarely lead to cutaneous symptoms. The classical systemic polyarteritis nodosa (PAN) is very rare. More frequent is cutaneous PAN presenting with a typical localized livedo racemosa and palpable subcutaneous nodules. The ANCA-associated vasculitides, which belong to the small vessel vasculitides, usually show systemic involvement and manifest on the skin with a diverse picture. Immune complex vasculitides are characterized by deposition of immunoglobulins on the walls of small vessels. The most common form is IgA vasculitis with the cardinal symptoms of palpable, round or oval and partially branched (retiform) purpura and a clear predilection for the legs. Serum disease is a príme example of systemic immune complex diseases due to large circulating immune complexes. Cryoglobulinemic vasculitis primarily involves small as well as medium sized vessels. Simultaneous involvement of vessels of different sizes is characteristic for vasculitis in systemic lupus erythematosus (SLE), Sjögren's syndrome, rheumatoid arthritis and sarcoidosis. Recurrent macular vasculitis in hypergammaglobulinemia is an episodic macular vasculitis of the small blood vessels. Bacteremic vasculitis of the small vessels (without direct microbial infection of the vessel wall) can typically also affect capillaries of dermal papillae and is usually accompanied by a disseminated intravascular coagulation (DIC). In some cases more than one distinct cutaneous vasculitis can occur in the same patient.
皮肤血管炎根据受累血管的大小和分布呈现出典型的临床症状。由于皮肤中没有大血管,巨细胞动脉炎和高安动脉炎很少导致皮肤症状。经典的系统性结节性多动脉炎(PAN)非常罕见。更常见的是皮肤型PAN,表现为典型的局限性网状青斑和可触及的皮下结节。属于小血管血管炎范畴的抗中性粒细胞胞浆抗体(ANCA)相关血管炎通常有全身受累情况,在皮肤上表现多样。免疫复合物性血管炎的特征是免疫球蛋白沉积在小血管壁上。最常见的形式是IgA血管炎,主要症状为可触及的圆形或椭圆形、部分呈分支状(网状)紫癜,且明显好发于双下肢。血清病是由于大量循环免疫复合物导致的系统性免疫复合物疾病的主要例子。冷球蛋白血症性血管炎主要累及小血管和中等大小血管。不同大小血管同时受累是系统性红斑狼疮(SLE)、干燥综合征、类风湿关节炎和结节病中血管炎的特征。高球蛋白血症性复发性黄斑血管炎是一种小血管的发作性黄斑血管炎。小血管菌血症性血管炎(血管壁无直接微生物感染)通常也可累及真皮乳头层的毛细血管,且常伴有弥散性血管内凝血(DIC)。在某些情况下,同一患者可能出现不止一种不同的皮肤血管炎。