Waldenström macroglobulinemia with an IgM-kappa antiepidermal basement membrane zone antibody.
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.


METHODS
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.


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.
Cobb
M W
MW
Department of Dermatology, National Naval Medical Center, Bethesda, MD 20889.
Domloge-Hultsch
N
N
Frame
J N
JN
Yancey
K B
KB
D001485:Basement Membrane / Q000737:chemistry*; D004817:Epidermis / Q000737:chemistry*; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis*; D007145:Immunoglobulin kappa-Chains / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D010266:Paraproteins / Q000032:analysis*; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology* / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000276:immunology* / Q000473:pathology
D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D010266:Paraproteins