Lupus erythematosus profundus with partial C4 deficiency responding to thalidomide
- 1 July 1991
- journal article
- case report
- Published by Oxford University Press (OUP) in British Journal of Dermatology
- Vol. 125 (1) , 62-67
- https://doi.org/10.1111/j.1365-2133.1991.tb06042.x
Abstract
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.Keywords
This publication has 26 references indexed in Scilit:
- Lupus Erythematosus PanniculitisMedical Clinics of North America, 1989
- C4 allotyping on plasma or serum: Application to routine laboratoriesHuman Immunology, 1988
- Phenotyping of human complement component C4, a class-III HLA antigenBiochemical Journal, 1986
- 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.BMJ, 1983
- The histopathology of lupus erythematosus panniculitisJournal of the American Academy of Dermatology, 1981
- Inherited structural polymorphism of the fourth component of human complement.Proceedings of the National Academy of Sciences, 1980
- Microdroplet Testing for HLA-A, -B, -C, and -D Antigens: The Philip Levine Award LectureAmerican Journal of Clinical Pathology, 1978
- GENETIC POLYMORPHISM IN HUMAN GLYCINE-RICH BETA-GLYCOPROTEINThe Journal of Experimental Medicine, 1972
- Lupus erythematosus panniculitis (profundus)Archives of Dermatology, 1971
- Panniculitis and systemic lupus erythematosusPublished by American Medical Association (AMA) ,1970