Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
J Dermatol. 2017 Nov;44(11):1276-1280. doi: 10.1111/1346-8138.13928. Epub 2017 Jun 23.
Telangiectasia macularis eruptiva perstans (TMEP) is a rare subtype of cutaneous mastocytosis, characterized by telangiectatic tan to brown macules on the trunk and extremities. Although TMEP has been descried as an uncommon disease in the literature, we often encounter patients with TMEP lesions in the outpatient clinic. We aimed to assess the clinical and histopathological characteristics of acquired bilateral TMEP, and the pathophysiological mechanism of acquired bilateral TMEP among these patients. We retrospectively reviewed 30 patients (28 men and 2 women) with acquired bilateral TMEP; multiple telangiectatic dark red to brown macules that were symmetrically distributed. The clinical characteristics and general histopathological findings of lesional skin were investigated. The number of mast cells was evaluated using immunohistochemical analysis with an antibody directed against c-kit (CD117). Acquired bilateral TMEP was predominantly localized on the sun-exposed area: the upper arm in 30 patients (100%), forearm in 19 patients (63.3%) and anterior chest in 15 patients (50%). A total of 16 patients (53.3%) showed at least one aggravating factor, including UV irradiation, alcohol use and heat exposure. Compared with the mast cell numbers in 19 age- and biopsy site-matched healthy controls (91 ± 29.0/mm ), the number of mast cells in the papillary dermal skin of acquired bilateral TMEP patients was significantly increased (159 ± 37.2/mm , P < 0.01). In addition, a significant difference in vessel numbers in the papillary dermis was observed between acquired bilateral TMEP patients and healthy controls (10.5 ± 1.9 vs 5.4 ± 1.0/mm , P < 0.01). Acquired bilateral TMEP is a relatively common disorder in middle-aged Asian men. An increased number of mast cells and dilated vessels might be a photoaging-related reactive process of chronic sun-exposure, which consequently leads to the formation of characteristic telangiectatic hyperpigmentary macules through certain melanogenic mediators.
持久性斑状毛细血管扩张性肥大细胞增多症（TMEP）是一种罕见的皮肤肥大细胞增多症亚型，其特征是躯干和四肢出现毛细血管扩张性的棕褐色斑疹。尽管 TMEP 在文献中被描述为一种罕见疾病，但我们在门诊经常会遇到 TMEP 病变的患者。我们旨在评估获得性双侧 TMEP 的临床和组织病理学特征，以及这些患者中获得性双侧 TMEP 的病理生理机制。我们回顾性分析了 30 例获得性双侧 TMEP 患者（28 名男性和 2 名女性）；多发性毛细血管扩张性暗红色至棕色斑疹，呈对称性分布。研究了皮损皮肤的临床特征和一般组织病理学发现。使用针对 c-kit（CD117）的抗体进行免疫组织化学分析来评估肥大细胞的数量。获得性双侧 TMEP 主要定位于暴露于阳光的区域：30 例患者（100%）的上臂、19 例患者（63.3%）的前臂和 15 例患者（50%）的前胸部。共有 16 例患者（53.3%）存在至少一种加重因素，包括紫外线照射、饮酒和热暴露。与 19 名年龄和活检部位匹配的健康对照者（91±29.0/mm ）的肥大细胞数量相比，获得性双侧 TMEP 患者的真皮乳头层中的肥大细胞数量显著增加（159±37.2/mm ，P＜0.01）。此外，还观察到获得性双侧 TMEP 患者与健康对照组之间真皮乳头层中血管数量存在显著差异（10.5±1.9 与 5.4±1.0/mm ，P＜0.01）。获得性双侧 TMEP 在中年亚洲男性中是一种相对常见的疾病。大量的肥大细胞和扩张的血管可能是与光老化相关的慢性暴露于阳光后的反应性过程，通过某些黑色素生成介质导致特征性的毛细血管扩张性色素斑的形成。
