A patient with IgG-kappa-associated subcorneal pustular dermatosis (Sneddon-Wilkinson disease) refractory to dapsone, etretinate, and plasma exchange was successfully treated with corticosteroids. A study of neutrophils from both blood and pustules was carried out before and during treatment. Levels of tumor necrosis factor-alpha were measured in serum, pustules, content, and supernatant of monocytes. The results suggest that a hyperactivation of neutrophils in the skin is due at least partly to excessive production of tumor necrosis factor-alpha.