Diffuse plane normolipaemic xanthomatosis in a patient with chronic lymphatic leukaemia and monoclonal gammopathy.

Diffuse plane normolipaemic xanthomatosis is a well-defined uncommon condition characterized by yellow-orange plaques involving the eyelids, neck, upper trunk, buttocks and flexural folds. Over half of the reported cases are associated with lymphoproliferative disorders. The condition may arise as a result of perivascular deposition of lipoprotein-immunoglobulin complexes. We present a female patient with clear clinical features of diffuse plane xanthomatosis associated with chronic lymphatic leukaemia and monoclonal gammopathy. In this patient, we found evidence of complement consumption supporting the hypothesis that complexes of lipoprotein and paraprotein are formed. Lipid electrophoresis of fasting serum revealed the presence of a faint precipitate at the application slot, also indicative of the presence of protein-lipid complexes. Copyright 2002 S. Karger AG, Basel