Clonal CD8(+) infiltration of the skin in common variable immunodeficiency: A prelymphomatous stage?

Common variable immunodeficiency is a heterogeneous syndrome characterized by hypogammaglobulinemia and recurrent bacterial infections. Patients with this syndrome also have an increased incidence of autoimmune disease and ma lignancy, most notably lymphoproliferative disorders. The treatment for com mon variable immunodeficiency is supported by the use of intravenous infusi on of immunoglobulins that allows for control of the disease and avoidance of recurrent opportunistic infections. This report describes a young patien t having common variable immunodeficiency who presented with a clonal CD8() lymphocytic infiltration of the skin clinically manifesting as a widespre ad papulonodular eruption and resolving with intravenous immunoglobulin the rapy We speculate that such a unique cutaneous presentation may represent a prelymphomatous condition because of the development of an autonomous T-su ppressor/cytotoxic cell clone, possibly also responsible for the impaired i mmunoglobulin production.