Diffuse normolipemic plane xanthoma associated with monoclonal gammopathy

Diffuse normolipemic plane xanthoma (DNPX) was first described by Altman and Winkelmann in 1962. It is a rare and non-inherited form of xanthomatosis. Clinically, the dermatosis is characterized by the presence of symmetric yellowish-orange plaques that favor the neck, upper trunk, flexural folds and periorbital region. It has been recognized to be associated with hematological diseases, especially with multiple myeloma and monoclonal gammopathy. We present a patient with diffuse plane xanthoma, normal lipid level, and monoclonal gammopathy.