Juvenile Xanthoma Multiplex: Case Report and Discussion of the Literature

Abstract

Since Rayer, in 1835, described a patient with “plaques jaunâtres des paupieres” a great variety of lesions have been reported which have come to be grouped under the general descriptive term “xanthoma.” Of these, a well recognized though relatively rare subgroup is known as “juvenile xanthoma multiplex.” The chief characteristic of this group is the occurrence, before or during puberty, of multiple, scattered cutaneous nodules of typical gross appearance and characteristic microscopic structure, associated usually with good general health, occasionally with xanthomatous lesions of other body structures, and probably always with certain underlying abnormalities of lipid metabolism. The condition is strikingly similar, clinically, to the more frequently observed adult variety, but like the adult form of xanthoma multiplex, it is to be differentiated from those forms of xanthoma which are associated with diabetes mellitus. The first of these juvenile cases of xanthoma multiplex to be reported was that of Virchow, in 1871. During the sixty years that have followed, 86 authors have reported some 120 cases, if we confine ourselves to the literature of Germany, France, Italy, Great Britain, and North America. Having had the opportunity of observing the development of one such case from its inception over a period of two years, the author has felt justified in presenting the following report and discussing it in the light of the literature bearing upon the problem of xanthoma.