Scleroderma, Sjögren-like Syndrome, and Chronic Graft-Versus-Host Disease

Abstract

A patient with acute myelogenous leukemia treated with an allogeneic bone marrow transplant developed acute graft-versus-host disease manifested by severe diarrhea, hepatitis, and a cutaneous eruption. As the graft-versus-host disease progressed to the chronic phase, the patient developed marked cutaneous sclerosis and symptoms of xerophthalmia and xerostomia. Biopsy of his indurated skin showed features of both graft-versus-host disease and scleroderma. Results of Schirmer's tests, corneal fluorescent studies, parotid flow-rate testing, and a lip biopsy were consistent with Sjögren's syndrome. Possibly, activated lymphocytes may have a role in the pathogenesis of graft-versus-host disease, scleroderma, and Sjögren's syndrome.