DISCOID LUPUS-ERYTHEMATOSUS IN AN X-LINKED CYTOCHROME-POSITIVE CARRIER OF CHRONIC GRANULOMATOUS-DISEASE

被引:18
作者
YEAMAN, GR [1 ]
FROEBEL, K [1 ]
GALEA, G [1 ]
ORMEROD, A [1 ]
URBANIAK, SJ [1 ]
机构
[1] ABERDEEN ROYAL INFIRM,DEPT DERMATOL,ABERDEEN AB9 2ZW,SCOTLAND
关键词
D O I
10.1111/j.1365-2133.1992.tb08405.x
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
A 13-year-old female presented with photosensitivity, recurrent aphthous ulcers and discoid lupus erythematosus (DLE)-like skin lesions. These symptoms have been linked to the carrier status of chronic granulomatous disease (CGD). Neutrophil (PMN) function was investigated by nitroblue tetrazolium reduction test and chemiluminescence. A severe impairment of PMN oxidative burst activity was revealed in spite of supranormal levels of cytochrome b245. Glucose-6-phosphate dehydrogenase activity was deficient. Her mother and two sisters also showed reduced PMN function. These findings are consistent with a cytochrome positive X-linked form of CGD with variable lyonization. DLE in association with the carrier status of this CGD variant has not been reported previously.
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页码:60 / 65
页数:6
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