Paller A S, Massey R B, Curtis M A, Pelachyk J M, Dombrowski H C, Leickly F E, Swift M
Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois.
J Pediatr. 1991 Dec;119(6):917-22. doi: 10.1016/s0022-3476(05)83043-4.
Ataxia-telangiectasia is a genetic syndrome with progressive cerebellar ataxia, oculocutaneous telangiectasias and other skin manifestations, variable immune system defects, chronic progressive sinopulmonary disease, and a high incidence of cancer. Cutaneous granulomas developed and persisted in eight patients with ataxia-telangiectasia, despite treatment with intravenously administered immune globulin, topical antibiotic therapy, and potent topical corticosteroid therapy. We were unable to identify an infectious agent; the granulomas may develop in an attempt to localize antigen in the presence of a dysfunctional immune system.
共济失调毛细血管扩张症是一种遗传性综合征，伴有进行性小脑共济失调、眼皮肤毛细血管扩张及其他皮肤表现、可变的免疫系统缺陷、慢性进行性鼻窦肺部疾病以及高癌症发病率。尽管接受了静脉注射免疫球蛋白、局部抗生素治疗和强效局部皮质类固醇治疗，但8例共济失调毛细血管扩张症患者仍出现并持续存在皮肤肉芽肿。我们未能识别出感染源；肉芽肿可能是在免疫系统功能失调的情况下，机体试图将抗原定位而形成的。
