Pyoderma gangrenosum is a necrotizing, ulcerative process commonly ass
ociated with inflammatory bowel disease but also occurring in several
other systemic illnesses. When associated with myelodysplastic syndrom
es, its clinical appearance is often atypical. We describe a patient w
ith severe, refractory bullous pyoderma gangrenosum. At autopsy cardia
c involvement was demonstrated, a finding not previously reported. Pat
ients with this unusual variant of pyoderma gangrenosum have a grave p
rognosis.