Waldenström macroglobulinemia with an IgM-kappa antiepidermal basement membrane zone antibody
Cobb, M.W.; Domloge-Hultsch, N.; Frame, J.N.; Yancey, K.B.
Archives of Dermatology 128(3): 372-376
1992
ISSN/ISBN: 0003-987X PMID: 1550370 Document Number: 400961
Waldenström macroglobulinemia, a lymphoplasmacytoid cell malignant neoplasm associated with a monoclonal IgM paraprotein, has been associated with a number of cutaneous manifestations. On rare occasions, IgM deposits have been demonstrated in the epidermal basement zone of patients with WM. We report the case of a patient with Waldenström macroglobulinemia and IgM-kappa paraprotein who had development of an eruption of pruritic papules and demonstrated the following unusual immunopathologic findings: (1) deposits of IgM-kappa in the epidermal basement membrane zone of lesional and nonlesional skin; (2) a circulating IgM-kappa antiepidermal basement membrane zone antibody; and (3) binding of this circulating IgM-kappa antiepidermal basement membrane zone antibody to both sides of 1 mol/L sodium chloride split skin. The cutaneous eruption cleared completely with oral psoralen with long-wave UV radiation in the A range (PUVA) therapy. We present a patient with Waldenström macroglobulinemia who had a distinctive papular eruption and immunopathologic findings suggesting that his paraprotein has specificity for the epidermal basement membrane zone.