Accelerated progressive systemic sclerosis in a patient with hypothenar hammer syndrome

We describe the first case of hypothenar hammer syndrome followed by accelerated systemic sclerosis (SSc). A sawmill worker with repeated wrist trauma presented with bilateral digital ischemia and fingertip ulcerations. Hypothenar hammer syndrome involving both hands was diagnosed based on classic angiographic studies showing occlusion of the ulnar arteries at the level of the hamate and occlusions of multiple digital arteries. He also had minimal digital swelling, telangiectatic lesions on the fingers, and a positive FANA test. Resected ulnar arteries showed clearcut changes compatible with both microtrauma and SSc. After surgical repair of both ulnar arteries, he rapidly developed severe SSc with significant cardiopulmonary involvement.