A case of non-Hodgkin's lymphoma following long-term corticosteroid therapy for Addison's disease

A 76-year-old Japanese woman had suffered from fatigue, weight loss, and cutaneous hyperpigmentation at the age of 38 years and was diagnosed as having tuberculous Addison's disease. Since then, corticosteroids had been administered effectively as hormonal replacement. At the age of 75 years, the patient presented with a progressive, painless swelling in the left eyelid due to an ill-defined tumor of rubbery consistency in the superotemporal aspect of the orbit. Computed tomography, magnetic resonance imaging, and scintigraphy revealed a wide distribution of tumors, but not in the adrenal gland, which led to the suspicion of systemic malignant lymphoma. Histopathologic examination of the excised orbital tumor was compatible with non-Hodgkin's lymphoma of the B-cell type. We believe this is the first report of Addison's disease presenting with non-Hodgkin's lymphoma. This disease process was characterized by the development of a lymphoid malignancy after long-term corticosteroid therapy to control the adrenal insufficiency, and by the widespread involvement of the lymph nodes and orbit but not the adrenal gland. Corticosteroid-induced abnormal immune state was considered to be the pathogenesis of this unusual complication.