Erythroderma with spongiotic dermatitis. Association with common variable hypogammaglobulinemia

Two middle-aged men presented with generalized erythroderma, diffuse alopecia, and hyperkeratosis of the palms and soles. Histopathologic study demonstrated sponglosis (epidermal intercellular edema) with a perivascular lymphohistiocytic infiltrate. Complete immunologic evaluation demonstrated that both patients had panhypogammaglobulinemia and markedly depressed in vitro pokeweed mitogen-induced Immunoglobulln secretion. One of the patients also showed poor lymphocyte responses In vitro to T cell mitogens and antigens and had a decreased ratio of helper to suppressor cells. In both patients, the cutaneous lesions Improved with systemic corticosterolds, but no significant alteration In the immunologic abnormalities was observed. This report Illustrates that chronic erythroderma may be the presenting clinical manifestation of common variable hypogammaglobulinemia.