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
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.
引用
收藏
页码:62 / 67
页数:6
相关论文
共 34 条
  • [1] GENETIC POLYMORPHISM IN HUMAN GLYCINE-RICH BETA-GLYCOPROTEIN
    ALPER, CA
    BOENISCH, T
    WATSON, L
    [J]. JOURNAL OF EXPERIMENTAL MEDICINE, 1972, 135 (01) : 68 - &
  • [2] INHERITED STRUCTURAL POLYMORPHISM OF THE 4TH COMPONENT OF HUMAN-COMPLEMENT
    AWDEH, ZL
    ALPER, CA
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1980, 77 (06): : 3576 - 3580
  • [3] BOLLING R, 1979, Z HAUTKRANKHEITEN, V54, P1081
  • [4] CERIMELE D, 1970, CHRON DERMATOL, V1, P295
  • [5] PRIMATE ERYTHROCYTE-IMMUNE COMPLEX-CLEARING MECHANISM
    CORNACOFF, JB
    HEBERT, LA
    SMEAD, WL
    VANAMAN, ME
    BIRMINGHAM, DJ
    WAXMAN, FJ
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1983, 71 (02) : 236 - 247
  • [6] LUPUS-ERYTHEMATOSUS PROFUNDUS - CASE REPORT ON AN AFRICAN PATIENT
    DOGLIOTTI, M
    HARTDEGEN, R
    SCHMAMAN, A
    [J]. DERMATOLOGICA, 1973, 147 (06): : 376 - 380
  • [7] DOR AM, 1982, SEM HOP PARIS, V58, P2847
  • [8] FAMILY STUDY OF THE MAJOR HISTOCOMPATIBILITY COMPLEX IN PATIENTS WITH SYSTEMIC LUPUS-ERYTHEMATOSUS - IMPORTANCE OF NULL ALLELES OF C4A AND C4B IN DETERMINING DISEASE SUSCEPTIBILITY
    FIELDER, AHL
    WALPORT, MJ
    BATCHELOR, JR
    RYNES, RI
    BLACK, CM
    DODI, IA
    HUGHES, GRV
    [J]. BMJ-BRITISH MEDICAL JOURNAL, 1983, 286 (6363): : 425 - 428
  • [9] LUPUS ERYTHEMATOSUS PROFUNDUS
    FOUNTAIN, RB
    [J]. BRITISH JOURNAL OF DERMATOLOGY, 1968, 80 (09) : 571 - &
  • [10] LUPUS PROFUNDUS IN CHILDREN - TREATMENT WITH HYDROXYCHLOROQUINE
    FOX, JN
    KLAPMAN, MH
    ROWE, L
    [J]. JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 1987, 16 (04) : 839 - 844