Epoprostenol is a vasodilator that is produced by vascular
endothelial cells and is currently the “gold standard” therapy for
patients with severe primary pulmonary hypertension or pulmonary
hypertension secondary to collagen vascular disease. Hypersensitivity
to the drug has not been reported. We report a case of a patient with
pulmonary hypertension and undifferentiated connective tissue disease
who, after 2 months of treatment with epoprostenol, presented with
rapidly progressive erythema, scaling, nausea and vomiting, and fever.
Test results from a skin biopsy specimen were consistent with a drug
reaction. The patient’ condition improved after rapid tapering of her
epoprostenol and administration of corticosteroids. Epoprostenol may be
associated rarely with severe erythroderma.