A 67-year-old woman had acutely diminished vision and painful proptosis of the left eye amidst a background of chronic headache, scalp tenderness, proximal myalgias, intermittent fever, anemia, and elevated erythrocyte sedimentation rates. All symptoms and signs were exquisitely corticosteroid-responsive. She underwent two negative temporal artery biopsies and several extensive negative evaluations for systemic disease. Ultimately deep scalp biopsy, bone biopsy, and bone marrow aspiration were performed, and all revealed a histologically well-differentiated lymphoma.

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