Acute graft-vs-host disease in an immunodeficient newborn possibly due to cytomegalovirus infection

Acute graft-vs-host disease occurring during the early weeks of life has been previously reported as a rare disease entity. We report a case of acute graft-vs-host disease in a female infant with an immunodeficiency that was thought to be secondary to intrauterine or neonatal cytomegalovirus infection or, less likely, to a severe combined immunodeficiency. The patient presented with a triad of failure to thrive, diarrhea, and maculopapular and petechial rash. The first clue to diagnosis was the skin biopsy finding of an epidermal lymphocytic infiltrate in association with individual necrotic keratinocytes. The diagnosis was confirmed at autopsy. In the absence of an obvious graft, the disease is believed to have been the result of maternofetal T-cell transfer in utero or at delivery.