XANTHOMATOSIS AND MONOCLONAL MYELOMATOUS GAMMAPATHY - ABOUT A CASE ALSO ASSOCIATED WITH SYSTEMIC AMYLOIDOSIS - REVIEW OF 42 CASES OF LITERATURE

  • 1 January 1979
    • journal article
    • research article
    • Vol. 106  (10) , 755-766
Abstract
A case of diffuse plane xanthomatosis associated with systemic amyloidosis and multiple myeloma at its outset is reported. Plane xanthomatosis is certainly an autonomous entity in comparison with systemic amyloidosis, for there are no amyloid deposits in xanthoma. The patient had .lambda. type Ig[immunoglobulin]G paraproteinemia, with Bence-Jones proteinuria. Lipid tests were considered as normolipemic, although some levels recall a type IV hyperlipoproteinemia. A review of literature about xanthomatosis-multiple myeloma was made. Two descriptions of the disease prevailed. When there is hyperlipoproteinemia, all clinical types of xanthomas may exist; multiple myeloma is generally typical (but sometimes not very progressive). When there is normolipidemia, the main clinical type is diffuse plane xanthomastosis; multiple myeloma is atypical and often only a monoclonal gammapathy is found. However, in both cases, the outstanding clinical type is diffuse plane xanthomatosis whether normo- or hyperlipemia. This indicates a possible underlying disease and multiple myeloma. Physiopathology still remains obscure, but there may exist immunologic disturbances in both cases.

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