Aoki Y, Miyake N, Yamasowa M, Inoue F, Takamatsu T, Mizumoto T, Okada T, Hirai M, Furukawa H
Department of Internal Medicine, Otsu Red Cross Hospital.
Rinsho Ketsueki. 1990 Mar;31(3):346-51.
A case of autoimmune hemolytic anemia (AIHA) and bullous pemphigoid (BP)-like skin lesion combined with Idiopathic thrombocytopenic purpura (ITP) is reported. A 25-years-old male, who had been diagnosed as ITP and treated at another hospital, was admitted in this hospital recently complaining of disseminated bullous-vesicular eruptions on the whole body and autoimmune hemolytic anemia. Examinations, disclosed that RBC was 364 X 10(4)/microliters, reticulocyte 40, platelet 3000/microliters, direct and indirect Coombs test positive, and platelet Coombs consumption test was positive leading to the diagnosis of AIHA and ITP, known as "Evans syndrome." Vesicular biopsy-findings and immunofluorescence study showed suspicion of BP, but clinical course and blister was not improved though the administration of prednisolone was performed. Reports of cases of BP complicated by Evans syndrome are very few. AIHA, ITP and BP are considered to have autoimmune disorders and their pathogenetic mechanism are discussed. This patient consulted another hospital one year later, when we heard that skin eruptions already had disappeared.
报告了一例自身免疫性溶血性贫血(AIHA)和类大疱性类天疱疮(BP)皮肤病变合并特发性血小板减少性紫癜(ITP)的病例。一名25岁男性,曾在另一家医院被诊断为ITP并接受治疗,近期因全身弥漫性大疱性水疱疹和自身免疫性溶血性贫血入住本院。检查发现红细胞为364×10⁴/微升,网织红细胞40,血小板3000/微升,直接和间接抗人球蛋白试验阳性,血小板抗人球蛋白消耗试验阳性,从而诊断为AIHA和ITP,即“伊文斯综合征”。水疱活检结果和免疫荧光研究提示疑似BP,但尽管给予泼尼松龙治疗,临床病程和水疱并未改善。BP并发伊文斯综合征的病例报道非常少。AIHA、ITP和BP被认为具有自身免疫性疾病,并对其发病机制进行了讨论。该患者一年后咨询了另一家医院,据我们所知皮肤疹已经消失。