LUPUS-ERYTHEMATOSUS PROFUNDUS WITH PARTIAL C4-DEFICIENCY RESPONDING TO THALIDOMIDE

被引：37
作者：
BURROWS, NP
WALPORT, MJ
HAMMOND, AH
DAVEY, N
JONES, RR
机构：
[1] ST THOMAS HOSP,ST JOHNS DERMATOL CTR,LONDON SE1 7EH,ENGLAND
[2] HAMMERSMITH HOSP,ROYAL POSTGRAD MED SCH,DEPT MED,RHEUMATOL UNIT,LONDON W12 0HS,ENGLAND
[3] HAMMERSMITH HOSP,ROYAL POSTGRAD MED SCH,DEPT IMMUNOL,LONDON W12 0HS,ENGLAND
来源：
BRITISH JOURNAL OF DERMATOLOGY | 1991年 / 125卷 / 01期
关键词：
D O I：
10.1111/j.1365-2133.1991.tb06042.x
中图分类号：
R75 [皮肤病学与性病学];
学科分类号：
100206 ;
摘要：
A female patient with disfiguring lupus erythematosus profundus (LEP) from the age of 13 years was found to have an isolated partial C4 deficiency, with reduced levels of both allotypes, C4A and C4B. A genetic basis for the hypocomplementaemia was confirmed by a family study of complement and HLA types which revealed heterozygous null alleles for C4A and C4B in the proband. Marked improvement in her cutaneous lesions occurred with thalidomide.
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页码：62 / 67
页数：6
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