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March 1992

Waldenström Macroglobulinemia With an IgM-? Antiepidermal Basement Membrane Zone Antibody

Author Affiliations


From the Department of Dermatology (Dr Cobb) and the Division of Hematology-Medical Oncology, Department of Medicine (Dr Frame), National Naval Medical Center, and the Department of Dermatology, Uniformed Services University of the Health Sciences (Drs Domloge-Hultsch and Yancey), Bethesda, Md.

Arch Dermatol. 1992;128(3):372-376. doi:10.1001/archderm.1992.01680130086011

• Background.—  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.

Observations.—  We report the case of a patient with Waldenström macroglobulinemia and IgM-κ paraprotein who had development of an eruption of pruritic papules and demonstrated the following unusual immunopathologic findings: (1) deposits of IgM-κ in the epidermal basement membrane zone of lesional and nonlesional skin; (2) a circulating IgM-κ antiepidermal basement membrane zone antibody; and (3) binding of this circulating IgM-κ 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.

Conclusions.—  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.(Arch Dermatol. 1992;128:372-376)

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