Oyefara B I, Kim H C, Danziger R N, Carroll M, Greene J M, Douglas S D
Division of Allergy, Immunology and Infectious Disease, Children's Hospital of Philadelphia, PA 19104, USA.
Clin Diagn Lab Immunol. 1994 Jan;1(1):38-43. doi: 10.1128/cdli.1.1.38-43.1994.
Chronic mucocutaneous candidiasis is an immunodeficiency disease characterized by T-cell dysregulation and chronic superficial candidal infections. We report on three patients with chronic mucocutaneous candidiasis who developed autoantibodies to erythrocytes. Our first patient, a 19-year-old female, developed autoimmune hemolytic anemia (AIHA) that required multiple courses of treatment, including corticosteroids, intravenous immunoglobulin, and danazol. During the last exacerbation of AIHA, intensive treatment with corticosteroids and intravenous immunoglobulin failed and yet the patient responded to plasmapheresis. Our second patient, a 21-year-old male, developed AIHA which responded to oral corticosteroid therapy. Our third patient, a 6-year-old female without evidence of hemolysis, was found to have erythrocyte autoantibodies on routine screening. These three patients had positive direct antiglobulin tests, and the first patient had both immunoglobulin G (IgG) and IgM erythrocyte autoantibodies, while the remaining two patients had only IgG autoantibody. This is the first report of the association of AIHA with chronic mucocutaneous candidiasis. We suggest that all patients with chronic mucocutaneous candidiasis be screened periodically for erythrocyte autoantibodies. Plasmapheresis, a safe ancillary procedure in the management of AIHA, may be life-saving in some cases. The occurrence of erythrocyte autoantibodies in mucocutaneous candidiasis may be related to immunoregulatory disorders in this disease.
慢性黏膜皮肤念珠菌病是一种免疫缺陷疾病，其特征为T细胞调节异常和慢性浅表念珠菌感染。我们报告了3例慢性黏膜皮肤念珠菌病患者，他们产生了抗红细胞自身抗体。我们的首例患者是一名19岁女性，患上了自身免疫性溶血性贫血（AIHA），需要多疗程治疗，包括使用皮质类固醇、静脉注射免疫球蛋白和达那唑。在AIHA的最后一次病情加重期间，强化使用皮质类固醇和静脉注射免疫球蛋白治疗失败，但患者对血浆置换有反应。我们的第二例患者是一名21岁男性，患上了AIHA，对口服皮质类固醇治疗有反应。我们的第三例患者是一名6岁女性，无溶血证据，在常规筛查中发现有红细胞自身抗体。这3例患者直接抗球蛋白试验均为阳性，首例患者同时有免疫球蛋白G（IgG）和IgM红细胞自身抗体，其余2例患者仅有IgG自身抗体。这是AIHA与慢性黏膜皮肤念珠菌病关联的首例报告。我们建议对所有慢性黏膜皮肤念珠菌病患者定期筛查红细胞自身抗体。血浆置换是AIHA治疗中的一种安全辅助程序，在某些情况下可能挽救生命。黏膜皮肤念珠菌病中红细胞自身抗体的出现可能与此病的免疫调节紊乱有关。
