Background: Waldenstrom macroglobulinemia is a plasma cell dyscrasia o
f undetermined cause characterized by the monoclonal proliferation of
lymphoplasmacytes in the bone marrow, lymph nodes, and spleen and elev
ated circulating levels and tissue deposition of monoclonal IgM produc
ed by these aberrant cells. Rarely, cutaneous manifestations of this d
isease have been reported. Observations: We report the case of a patie
nt with bullous dermatosis induced by Waldenstrom macroglobulinemia an
d demonstrate the subepidermal location of the separation and the pres
ence of IgM and kappa light chains by immunoperoxidase, immunofluoresc
ent techniques, and electron microscopy with immunogold staining. Immu
noblotting revealed a strong band at the 290-kd area. Conclusions: The
demonstration of the separation in the upper dermis at the site of Ig
M deposits suggests that these deposits may be an etiologic factor in
this rare manifestation.