Frequency of the carrier state for X-linked chronic granulomatous disease among females with lupus erythematosus.
Carriers for chronic granulomatous disease (CGD) and patients with lupus erythematosus (LE) share several characteristics: They are mostly females, they reduce nitroblue tetrazolium (NBT) poorly in their neutrophils, and, in some cases, they have similar skin lesions. We thus investigated 19 female LE patients for the presence of laboratory findings characteristic of the carrier state for CGD. None of these patients turned out to have the combined abnormality of neutrophil bactericidal activity and neutrophil NBT-reduction that is diagnostic of CGD carrier state in the X-linked form. An increased frequency of CGD carriers among female LE patients thus appear to be unlikely. Why some CGD carriers develop skin lesions typical of LE remains unexplained.
D000328:Adult; D000368:Aged; D001770:Blood Bactericidal Activity; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000235:genetics; D008875:Middle Aged; D009504:Neutrophils / Q000502:physiology; D010585:Phagocyte Bactericidal Dysfunction / Q000235:genetics*; D012730:Sex Chromosomes