Xanthomatosis resembling scleroderma in multiple myeloma

A patient with multiple myeloma had severe cutaneous and gastrointestinal xanthomatization and symptoms characteristic of systemic sclerosis. Clinical findings included thickened sclerotic skin, dysphagia associated with an akinetic esophagus, and abnormal findings on roentgenograms of the upper and lower gastrointestinal tract. Biopsy specimens obtained from the skin and the gastrointestinal tract studied by light and electron microscopy showed thickening, hyalinization, and xanthomatization of the subepithelial connective tissue. Macrophages containing lipid vacuoles were shown to assume a perivascular orientation on ultrastructural study. Our case appears to be unique since this combination of findings has not been reported previuosly. In addition, our electron micrographs may help to define better the pathogenetic process involved in the association between myeloma and plane xanthomata.