Turchin Irina, Barankin Benjamin, Schloss Eric
Faculty of Medicine, University of Calgary, Calgary, Canada.
Int J Dermatol. 2006 Oct;45(10):1215-7. doi: 10.1111/j.1365-4632.2006.02648.x.
Mastocytosis is a heterogeneous group of disorders characterized by mast cell hyperplasia in the bone marrow, liver, spleen, lymph nodes, gastrointestinal tract, and skin. We present a patient with malignant mastocytosis of 11 years' duration. This case highlights the cutaneous findings of mastocytosis with systemic involvement, yet the patient maintains a relatively normal lifestyle with only minimal discomfort and only borderline normochromic anemia. Thus his course is not truly that of malignant mastocytosis but of indolent systemic mastocytosis with cutaneous findings of telangiectasia macularis eruptiva perstans (TMEP).
肥大细胞增多症是一组异质性疾病，其特征为骨髓、肝脏、脾脏、淋巴结、胃肠道和皮肤中肥大细胞增生。我们报告一例病程长达11年的恶性肥大细胞增多症患者。该病例突出了系统性受累的肥大细胞增多症的皮肤表现，然而患者维持相对正常的生活方式，仅有轻微不适且仅有轻度正色素性贫血。因此，他的病程并非真正的恶性肥大细胞增多症，而是具有持久性斑疹性毛细血管扩张（TMEP）皮肤表现的惰性系统性肥大细胞增多症。
