Discoid lupus erythematosus-like lesions in carriers of X-linked chronic granulomatous disease.
A questionnaire was sent to 16 carriers of the X-linked cytochrome-b558 negative variant of chronic granulomatous disease (CGD). Of the 15 who answered the questionnaire and from data of one additional case, 70% reported recurrent aphthous stomatitis and 63% had recurrent skin eruptions. Five of the carriers (31%) had clinically discoid lupus erythematosus (DLE), although the histopathology was not typical and the immunofluorescence findings were negative. The infiltrate in the lesional skin of three of these patients was analysed using monoclonal antibodies against CD3, CD4, CD8, CDIa, HLA-DR, CDIc, interdigitating cells, B lymphocytes and cells of the monocyte/macrophage lineage. The immunophenotype of the infiltrating cells resembled that found in discoid lupus erythematosus.
Sillevis Smitt
J H
JH
Department of Dermatology, Academic Medical Center, Amsterdam, The Netherlands.
Weening
R S
RS
Krieg
S R
SR
Bos
J D
JD
D000293:Adolescent; D000328:Adult; D002353:Carrier State; D005260:Female; D008040:Genetic Linkage; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008179:Lupus Erythematosus, Discoid / Q000473:pathology*; D008875:Middle Aged; D010641:Phenotype; D012867:Skin / Q000276:immunology / Q000473:pathology*; D013281:Stomatitis, Aphthous / Q000150:complications; D014960:X Chromosome