Successful treatment of toe gangrene with moderate-dose steroid therapy in a patient with systemic sclerosis (CREST syndrome)

A 68-year-old woman first noticed Raynaud's phenomenon at the age of 35, and she was diagnosed as having systemic sclerosis (SSc) because of cutaneous calcinosis, sclerodactyly, telangiectasia, sausage-like finger, digital pitting scar in 1971. In October, 1994, she was suffered from cold sensation in her right foot, and on November 2 she noticed the painful ulcers on the right toe, which progressed to be gangrenous. Every antiplatelet or thrombolytic agents including PGE₁, argatroban, sarpogrelate hydrochloride and urokinase were unsuccessful. Angiograms revealed marked narrowing of bilateral anterior and posterior tibial and peroneal arteries. Prednisolone 30 mg/day was started, which resulted in successful response within 10 days. Large vessel involvement in SSc patients has been reported to be rare, and its treatment remains to be established. We believe our case help understanding the pathophysiology of such rare manifestation in SSc.