DIFFUSE PLANE XANTHOMA - CLINICOPATHOLOGICAL STUDY OF 8 CASES

Background: Diffuse plane xanthoma (DPX) usually has been described in association with a reticuloendothelial disease. However the incidence of an underlying disease and its relation to clinical and histopathol ogic features have not been previously described. Objective: We descri be the clinicopathologic features of a series of patients with DPX. Me thods: Eight patients with DPX were diagnosed and studied between 1987 and 1996. Results: Three of the 8 patients had a reticuloendothelial disease (benign monoclonal gammopathy in 2 and chronic myelomonocytic leukemia in 1). These 3 patients had larger cutaneous lesions and invo lvement of trunk and extremities. Histologic examination showed only f oamy macrophages in 2 of the 8 patients. The remainder exhibited macro phages, foamy histiocytes, lymphocytes, and Touton cells. Conclusion: The incidence of underlying disease associated with DPX seems to be lo wer than expected. However, patients with DPX should be observed for t he potential occurrence of an associated condition.