Krupnick A I, Shim H, Phelps R G, Cunningham-Rundles C, Sapadin A N
Department of Dermatology, Box 1047, Mount Sinai School of Medicine, One East 100th Street, New York, NY 10029, USA.
Mt Sinai J Med. 2001 Sep-Oct;68(4-5):326-30.
Combined immunodeficiency disorders are characterized by abnormalities in cellular and humoral immunity. This classification includes common variable immunodeficiency (CVI), a primary immunodeficiency disorder characterized by hypogammaglobulinemia, recurrent bacterial infections, and significant T-cell abnormalities. Associated autoimmune diseases include rheumatoid arthritis, pernicious anemia, idiopathic thrombocytopenic purpura, and systemic lupus erythematous. Granulomatous lesions in lymphoid tissues, solid organs, and skin have been reported. We describe a patient with CVI who developed cutaneous granulomas with perineural invasion; to our knowledge, this is a previously undescribed feature.
联合免疫缺陷病的特征是细胞免疫和体液免疫异常。该分类包括常见变异型免疫缺陷（CVI），这是一种原发性免疫缺陷病，其特征为低丙种球蛋白血症、反复细菌感染以及明显的T细胞异常。相关的自身免疫性疾病包括类风湿性关节炎、恶性贫血、特发性血小板减少性紫癜和系统性红斑狼疮。已有报道在淋巴组织、实体器官和皮肤中出现肉芽肿性病变。我们描述了一名患有CVI的患者，其出现了伴有神经周围侵犯的皮肤肉芽肿；据我们所知，这是一个此前未被描述过的特征。
