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Diffuse plane xanthomatosis associated with a monoclonal band displaying anti‐smooth muscle antibody activity

We report a patient with diffuse planar xanthomalous lesions associated with a benign monoclonal gammopathy. This patient has a very high litre of anti‐smooth muscle antibody (1:10,240), but normal liver function tests and a normal liver biopsy. The paraprotein band displays anti‐smooth muscle antibody activity. There is no evidence of any other underlying disease process. The findings are consistent with a diagnosis of diffuse plane xanthomatosis, but the mechanism of xanthoma formation remains unclear.