Kragballe K, Borregaard N, Brandrup F, Koch C, Staehrjohansen K
Clin Exp Immunol. 1981 Feb;43(2):390-8.
Out of fifteen carriers of X-linked chronic granulomatous disease five had discoid lupus erythematosus-like skin lesions together with recurrent aphthous-like stomatitis, another five had only recurrent aphthous-like stomatitis, and the remainder were symptom-free. In individual carriers monocytes and neutrophils were equally reduced in their capacity for superoxide production, [1-14C]glucose oxidation and antibody-dependent cytotoxicity; but within he group of carriers a broad spectrum of depression was found. The degree of depression was closely related to the manifestations of clinical disease. It is suggested that the defective oxygen-dependent metabolism might play an aetiological role in the development of inflammatory diseases in carriers of chronic granulomatous disease. Two out of 10 unselected females with discoid lupus erythematosus were shown to be carriers of X-linked chronic granulomatous disease. Screening for this carrier state might therefore be of importance in these patients.
在15名X连锁慢性肉芽肿病携带者中，5人有盘状红斑狼疮样皮肤损害并伴有复发性口疮样口炎，另外5人仅有复发性口疮样口炎，其余则无症状。在个体携带者中，单核细胞和中性粒细胞产生超氧化物的能力、[1-¹⁴C]葡萄糖氧化能力及抗体依赖性细胞毒性均同等程度降低；但在携带者群体中发现了广泛的功能抑制范围。功能抑制程度与临床疾病表现密切相关。提示氧依赖性代谢缺陷可能在慢性肉芽肿病携带者炎症性疾病的发生中起病因学作用。在10名未经选择的盘状红斑狼疮女性患者中，有2人被证明是X连锁慢性肉芽肿病携带者。因此，对这些患者进行这种携带者状态的筛查可能具有重要意义。
