Here a patient, a 78-year-old woman, with progressive toe necrosis and local severe pain due to collagen disease was presented. The case showed no reaction with various treatments on the guideline for vascular lesions combined with collagen disease. Then oral administration of sildenafil was indicated and significantly suppressed the local severe pain and progress of the necrosis. Then the leg and foot was preserved, and the patient could depart on foot from the hospital. It is suggested that sildenafil is effective for refractory cases due to vascular lesions of collagen diseases. However, although public indication of sildenafil is fixed in pulmonary hypertension, in this time the application for the patient without the sign is currently off-label use. Therefore, the administration should be limited to refractory patients to conventional therapies. Sufficient informed consent should be established. Medical stuffs should pay great attention to the patient during the treatment.
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