Subcorneal pustulosis with combined lack of IgG/IgM and monoclonal gammopathy type IgA/Kappa.

Subcorneal pustulosis (Sneddon-Wilkinson disease) is a rare inflammatory neutrophilic dermatosis. While subcorneal pustulosis is often associated with an IgA gammopathy, the combined lack of IgG/IgM seen in our case is rare. An 83-year-old man with combined lack of IgG/IgM and monoclonal gammopathy type IgA/Kappa presented with subcorneal pustules. Intravenous immunoglobulin therapy led to complete regression and might be another therapeutic option.