A 71 -year-old woman had a 7-month history of multiple asymptomatic papules on the upper part of the body. The clinical picture and histopathologic findings ranfirmed the diagnosis of xanthoma disseminatum. Further studies revealed Waldenstrom's macroglobulinemia. Treatment with chlorambucil and prednisone led to a dramatic reduction in her paraprotein level. In addition, cutaneous lesions improved and new lesions stopped appearing. Treatment of selected papules by cryotherapy or intralesional corticosteroid injection provided further improvement. This case is notable because (1) it is the first report of xanthoma disseminatum in a patient with Waldenström's macroglobulinemia and (2) there was an unusually good response to therapy.Le texte complet de cet article est disponible en PDF.