Pyoderma gangrenosum is a necrotizing, ulcerative process commonly associated with inflammatory bowel disease but also occurring in several other systemic illnesses. When associated with myelodysplastic syndromes, its clinical appearance is often atypical. We describe a patient with severe, refractory bullous pyoderma gangrenosum. At autopsy cardiac involvement was demonstrated, a finding not previously reported. Patients with this unusual variant of pyoderma gangrenosum have a grave prognosis.