Severe Erythroderma as a Complication of Continuous Epoprostenol Therapy

Epoprostenol is a vasodilator that is produced by vascularendothelial cells and is currently the “gold standard” therapy forpatients with severe primary pulmonary hypertension or pulmonaryhypertension secondary to collagen vascular disease. Hypersensitivityto the drug has not been reported. We report a case of a patient withpulmonary hypertension and undifferentiated connective tissue diseasewho, after 2 months of treatment with epoprostenol, presented withrapidly progressive erythema, scaling, nausea and vomiting, and fever. Test results from a skin biopsy specimen were consistent with a drugreaction. The patient’ condition improved after rapid tapering of herepoprostenol and administration of corticosteroids. Epoprostenol may beassociated rarely with severe erythroderma.