A 20-year-old woman was admitted to our hospital because of bilateral pretibial edema. Administration of prednisolone was started after she was diagnosed with systemic lupus erythematosus (SLE). However, skin ulcers on her extremities developed; they subsequently worsened with tapering of prednisolone. She also developed pulmonary hypertension (PH). Her skin ulcers improved considerably after administration of bosentan, an endothelin receptor antagonist. Bosentan may be efficacious not only for PH but also for refractory skin ulcers.