Acral lesions in tuberous sclerosis complex: Insights into pathogenesis
Acral lesions in tuberous sclerosis complex: Insights into pathogenesis
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DOI：
10.1016/j.jaad.2009.08.042
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发表时间：
2010-08-01
影响因子：
13.8
通讯作者：
Darling, Thomas N.
中科院分区：
文献类型：
--
作者：
Aldrich, Shelley L.;Hong, Chien-Hui;Darling, Thomas N.
背景：结节性硬化症（TSC）患者易发生甲周纤维瘤及其他肢端病变。
目的：我们试图确定结节性硬化症患者肢端皮肤病变的数量、类型和位置。
方法：我们对76名成年女性结节性硬化症患者进行了检查并拍照。
结果：患者年龄在20至69岁之间，平均年龄为39±11岁。76名患者中有61名（80%）观察到甲周纤维瘤。甲周纤维瘤比甲下纤维瘤更常见，足部比手部更常见，且在第五趾出现的频率最高。指甲上的纵行沟可伴有或不伴有可见的纤维瘤。在22名患者（29%）中观察到纵向的红色短条纹——我们称之为“红色彗星”的病变。在14名患者（18%）中观察到纵向白甲。1名患者有孤立性指（趾）过度生长，1名患者有厚皮性骨膜病。
局限性：本研究未包括男性或儿童。
结论：对结节性硬化症患者皮肤病变的检查除了甲周纤维瘤外，还应包括对指甲纵行沟、红色彗星、纵向白甲和裂片形出血的检查，这样可以得到改进。结节性硬化症甲周纤维瘤的解剖分布不是随机的，似乎与创伤促进的肿瘤形成相符。（《美国皮肤病学会杂志》2010年；63:244 - 251）
Background: Patients with tuberous sclerosis complex (TSC) are predisposed to developing ungual fibromas and other acral lesions.Objective: We sought to determine the numbers, types, and locations of acral skin lesions in TSC.Methods: We examined and photographed 76 adult women with TSC.Results: The age of the patients ranged from 20 to 69 years, with a mean age of 39 +/- 11 years. Ungual fibromas were observed in 61 of 76 patients (80%). Periungual fibromas were more common than subungual fibromas, were more common on the feet than the hands, and showed the greatest frequency on the fifth toe. Longitudinal grooves in the nails occurred with or without a visible fibroma. Longitudinal short red streaks-lesions that we term "red comets"-were observed in 22 patients (29%). Longitudinal leukonychia was observed in 14 patients (18%). One patient had isolated digital overgrowth and one patient had pachydermodactyly.Limitations: No men or children were included in this study.Conclusions: Examination of patients for skin lesions of TSC could be improved by including inspection for longitudinal nail grooves, red comets, longitudinal leukonychia, and splinter hemorrhages in addition to Lingual fibromas. The anatomic distribution of TSC ungual fibromas is not random and appears consistent with trauma-promoted tumor formation. (J Am Acad Dermatol 2010;63:244-51.)