A patient with systemic lupus erythematosus of long duration developed secondary amyloidosis and finally died after the additional complication of malignant lymphoproliferative disease. Multiple system involvement, typical serologic findings, and postmortem evidence substantiated the diagnosis of lupus erythematosus. Amyloid deposition was found in several organs, but was notably extensive in the adrenal cortex. This extensive deposition resulted in adrenal insufficiency, which was diagnosed clinically and treated until the patient's death from lymphoma. The particular nature of the malignant lymphoma is emphasized; a distinctive feature was the disappearance of positive lupus erythematosus cells from the buffy coat and the reduction in titers of relevant serologic tests toward the end of the illness. In spite of this reduction, many hematoxylin bodies and abundant complete lupus er- T h i s report describes a patient with long-standing systemic lupus erythematosus w h o developed adrenal insufficiency secondary t o amyloidosis. Several m o n t h s later t h e patient developed a n d died from complications of a n unusual malignant lymphoma. Postmortem examination demonstrated the presence of hematoxylin bodies and LE cells in t h e lungs.
CASE REPORTThe patient was a 65-year-old Mexican male, admitted to the hospital for nausea, abdominal pain, weakness, and dizziness on February 8, 1970. The patient also complained of pain, stiffness, and swelling of his wrists, elbows, shoulders, and ankles. His history revealed periodic exacerbations and remissions also involving the metatarsal phalangeal joints and metacarpal phalangeal joints, neck stiffness, and pain and stiffness in the sacrolumbar region since 1962. In 1960 he was comatose for several days, but no specific diagnosis was made. There was no history of ingestion of procainamide, hydralazine, isoniazid, dilantin, phenothiazines, or other drugs that could be associated with the development of a lupus-like illness.Physical examination on admission in 1970 revealed a supine blood pressure of 120/60 mm Hg, pulse 72 and regular. On standing, the blood pressure dropped to 80/60 mm Hg and the pulse rose to 100. There was patchy scalp alopecia. The skin areas behind his ears, neck, and the creases of his arms and hands were hyperpigmented. Gynecomastia was noted.