Telangiectasia macularis eruptiva perstans in polycythemia rubra vera

We describe a 65-year-old Japanese man with a 20-year history of telangiectasia macularis eruptiva perstans, who developed polycythemia rubra vera and duodenal ulcer 10 and 12 years respectively after the onset of mastocytosis. Involvement of mast cells was found in neither bone marrow nor gastrointestinal tract. Immunohistochemical staining revealed that the mast cell was positive for both tryptase and chymase, indicating the nature of cutaneous mast cells. Despite the coexistence of a hematologic disorder, our case is suggested to have cutaneous but not systemic mastocytosis presenting as telangiectasia macularis eruptiva perstans.