Cases from the Osler Medical Service at Johns Hopkins University

Abstract

PRESENTING FEATURES: A 36-year-old African American woman was admitted with a history of chills, fever, productive cough, and malaise of several days' duration, as well as an increase in baseline dyspnea on exertion. Her past medical history was notable for hemodialysis-dependent end-stage renal disease due to hypertension. Two years before admission, she successfully underwent angioplasty for a right subclavian vein stenosis due to a dialysis catheter. Nine months before admission, she had a new arteriovenous fistula placed in her right forearm because the fistula in her left arm had repeated complications, including pseudoaneurysm formation and thrombosis. Since placement of the new fistula, she noticed a steady, gradual increase in the size of her right breast and right upper arm. She also slowly developed worsening dyspnea on exertion, as well as back pain associated with her enlarged breast. A mammogram performed 3 months before admission revealed substantial vascular and lymphatic engorgement in the right breast but no distinct mass. The left breast had similar, although less dramatic, changes, as well as a 2.0- × 1.5-cm mass in the left lower quadrant. On physical examination, the patient was febrile (103.9 oF), with a heart rate of 123 beats per minute, blood pressure of 192/114 mm Hg, and oxygen saturation of 99% on room air. She was a moderately obese woman in mild distress. Her left breast was dysplastic. Her right breast was massively enlarged (Figure 1), about eight times the size of her left breast, with peau d'orange skin changes (Figure 2). There were no palpable masses in either breast. Numerous large subcutaneous veins were visible over her right breast, right arm, upper abdomen, and posterior chest. She had posterior cervical subcentimeter lymphadenopathy with no palpable axillary lymph nodes. Breath sounds were markedly diminished in the lower half of her right lung field with dullness to percussion in this area. Heart sounds were normal with an elevated jugular venous pressure to the level of the jaw. Her abdomen was benign. Extremities were normal except for an edematous, enlarged right arm. Neurologic examination was normal. Laboratory studies were normal except for an elevated blood urea nitrogen concentration and creatinine levels that were consistent with chronic renal failure. A chest radiograph showed a moderate right pleural effusion and small left pleural effusion. Chest computed tomographic (CT) scan with contrast confirmed these findings and also revealed dependent atelectasis overlying both pleural effusions, asymmetrical thickening, enlargement of soft tissue in the right breast, multifocal nodular opacities in bilateral lung fields, and dilation of the internal mammary vein, azygous venous system, and chest wall veins. A right thoracentesis revealed transudative fluid with negative cultures and normal cytopathology. What is the diagnosis?