The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency
The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency
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DOI：
10.1016/s1081-1206(10)62152-7
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发表时间：
2003-02-01
影响因子：
5.9
通讯作者：
Stiehm, ER
中科院分区：
文献类型：
--
作者：
Kalfa, VC;Roberts, RL;Stiehm, ER
背景：大多数患有慢性皮肤粘膜念珠菌病（CMC）的患者具有针对白色念珠菌的细胞介导免疫的选择性缺陷（如皮肤无反应性和对念珠菌抗原的淋巴增殖反应减少所证明）和完整的抗体反应。许多 CMC 患者还出现其他生物体感染，表明存在更广泛的免疫缺陷。 目的：本研究的目的是描述一名患有 CMC 和选择性抗体缺陷的患者，并确定 8 名先前报告的类似患者。数据来源：使用通过搜索 MEDLINE 数据库得出的相关英文文章。结果：我们描述了一名 18 岁男性患者，他在婴儿时就被确诊患有 CMC，后来出现了免疫球蛋白 (Ig)G2、IgG4 和 IgA 12 岁时的缺陷与疫苗抗原的抗体反应较差有关。我们已经确定了另外八名先前报告的患有选择性抗体缺陷和细菌感染的 CMC 患者。所有 9 名患者均存在 IgG2 缺乏，其中 8 名患者与 IgG4 缺乏相关，3 名患者与 IgA 缺乏相关。六名患者对肺炎球菌多糖疫苗的抗体反应较差或缺乏，所有九名患者均出现严重的复发性肺部感染。结论：我们建议这些病例代表了 CMC 的独特表型，应研究常见的组织相容性白细胞抗原类型和分子缺陷。
Background: Most patients with chronic mucocutaneous candidiasis (CMC) have a selective defect of cell-mediated immunity against Candida albicans (as demonstrated by cutaneous anergy and decreased lymphoproliferative responses to Candida antigen) and intact antibody responses. Many CMC patients also develop infections with other organisms, suggesting a more extensive immunologic defect.Objectives: The aim of this study was to describe a patient with CMC and selective antibody deficiency and identify eight similar previously reported patients.Data Sources: Relevant articles in the English language derived from searching the MEDLINE database were used.Results: We describe an 18-year-old male patient who was identified with CMC as an infant and later developed immunoglobulin (Ig)G2, IgG4, and IgA deficiency at age 12 associated with poor antibody responses to vaccine antigens. We have identified eight other previously reported CMC patients with selective antibody deficiencies and bacterial infections. IgG2 deficiency was present in all nine patients, and was associated with IgG4 deficiency in 8 patients and IgA deficiency in 3 patients. Six patients had poor or absent antibody responses to pneumococcal polysaccharide vaccine, and all nine patients developed severe recurrent lung infections.Conclusions: We suggest that these cases represent a distinct phenotype of CMC and should be studied for common histocompatibility leukocyte antigen types and molecular defects.