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Case Reports
. 1992 Jan;126(1):60-5.
doi: 10.1111/j.1365-2133.1992.tb08405.x.

Discoid lupus erythematosus in an X-linked cytochrome-positive carrier of chronic granulomatous disease

G R Yeaman  1 , K Froebel, G Galea, A Ormerod, S J Urbaniak
Affiliations
Case Reports

Discoid lupus erythematosus in an X-linked cytochrome-positive carrier of chronic granulomatous disease

G R Yeaman et al. Br J Dermatol. 1992 Jan.

Abstract

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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