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C1 inhibitor deficiency simulating systemic lupus erythematosus
Author(s) -
SHIRAISHI S.,
NARA Y.,
WATANABE Y.,
MATSUDA K.,
MIKI Y.
Publication year - 1982
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1982.tb04540.x
Subject(s) - medicine , betamethasone , systemic reaction , systemic disease , immunology , complement (music) , lupus erythematosus , complement system , dermatology , systemic administration , antibody , immunopathology , allergy , biology , biochemistry , microbiology and biotechnology , complementation , in vivo , gene , phenotype
SUMMARY A 48–year‐old Japanese woman with systemic lupus erythematosus‐like lesions of the skin and lips was found to have hereditary angio‐oedema. Complement studies revealed low CH5O, C19, C4 and C1 inhibitor levels, with normal C3 and C5 levels. Dramatic clinical improvement followed fresh normal human blood transfusion and systemic betamethasone administration, while the deficient complement component levels were unchanged.