Suzuki Kenji, Konishi Noriko, Tokura Yoshiki, Takigawa Masahiro
Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handa-yama, Hamamatsu 431-3192, Japan.
Eur J Dermatol. 2002 Mar-Apr;12(2):201-3.
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.
我们描述了一名65岁的日本男性，他患有持久性斑疹性毛细血管扩张症20年，分别在肥大细胞增多症发病后10年和12年出现真性红细胞增多症和十二指肠溃疡。在骨髓和胃肠道均未发现肥大细胞浸润。免疫组织化学染色显示肥大细胞对类胰蛋白酶和糜蛋白酶均呈阳性，表明为皮肤肥大细胞的特性。尽管存在血液系统疾病，但我们的病例提示为表现为持久性斑疹性毛细血管扩张症的皮肤型而非系统性肥大细胞增多症。
