Wuepper K D, Watson P A, Kazmierowski J A
J Invest Dermatol. 1980 May;74(5):368-71. doi: 10.1111/1523-1747.ep12543789.
Early cutaneous lesions of erythema multiforme or mucosal lesions of the Stevens-Johnson syndrome contain delicate granular deposits of immune reactants and/or complement components lodged in the walls of vessels of the papillary dermis. Such deposits are not present in normal, unaffected skin although they can be caused to occur there by injection of substances which increase vascular permeability. Factors which cause accumulation of mononuclear cells in the papillary dermis have not been elucidated but we suggest that receptors for the C3d fragment of the third component of complement could be responsible. Factors which cause intercellular edema in the epidermis, epidermal-dermal separation, or necrotic keratinocytes are unknown. We, and others, may now measured immune complexes in the serum of patients experiencing these reaction patterns by means of Raji cell radioimmunoassay, C1q precipitation, 125I-C1q binding, cryoprecipitation and with monoclonal rheumatoid factor. Cryoimmunoglobulins have also been described and antigens of Herpesvirus hominus have been measured in the cryoprecipitate of 2 patients who experienced erythema multiforme following recurrent herpesvirus infection. Erythema multiforme commonly accompanied serum sickness reactions which occurred following injection of immune horse serum in the 1980s (von Pirquet and Schick). Such reactions are now well established as due to circulating immune complexes in antigen excess.
多形红斑的早期皮肤损害或史蒂文斯-约翰逊综合征的黏膜损害含有免疫反应物和/或补体成分的细微颗粒状沉积物，沉积于乳头层真皮的血管壁。正常未受累皮肤中不存在此类沉积物，不过通过注射可增加血管通透性的物质能使其在正常皮肤中出现。导致单核细胞在乳头层真皮中积聚的因素尚未阐明，但我们认为补体第三成分C3d片段的受体可能起作用。导致表皮细胞间水肿、表皮与真皮分离或角质形成细胞坏死的因素尚不清楚。我们及其他研究人员现在可以通过拉吉细胞放射免疫测定、C1q沉淀、125I-C1q结合、冷沉淀以及单克隆类风湿因子来检测出现这些反应模式的患者血清中的免疫复合物。也有关于冷球蛋白的描述，并且在2例复发性疱疹病毒感染后出现多形红斑的患者的冷沉淀物中检测到了人疱疹病毒的抗原。多形红斑常伴有血清病反应，在20世纪80年代注射免疫马血清后会出现这种情况（冯·皮尔凯和希克）。现在已明确此类反应是由于抗原过量时循环免疫复合物所致。
