Premium
Complement abnormalities in diffuse plane xanthomatosis with paraproteinaemia
Author(s) -
JONES R. RUSSELL,
BAUGHAN A.S.J.,
CREAM J.J.,
LEVANTINE A.,
WHICHER J.T.
Publication year - 1979
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.1365-2133.1979.tb05652.x
Subject(s) - complement (music) , medicine , chemistry , complementation , gene , phenotype , biochemistry
SUMMARY Paraproteinaemia may be associated with xanthomatous skin deposits and these can arise in the absence of elevated lipid levels. Two cases of benign monoclonal gammopathy with diffuse plane xanthomatosis are reported. Case 1 exhibited hypolipidaemia and a functional deficiency of C1 esterase inhibitor. Case 2 showed a normal lipoprotein profile, abnormal platelet aggregation, and a cutaneous vasculitis with evidence of complement consumption via the classical pathway. The significance of these abnormalities is discussed.