Krakowski Andrew C, Nguyen Tuyet A
Pediatrics. 2015 Sep;136(3):e709-13. doi: 10.1542/peds.2015-0025.
Angiofibromas are the most recognized cutaneous manifestations of tuberous sclerosis complex. Angiofibromas can be associated with disfigurement, bleeding, pruritus, and erythema and may lead to significant psychosocial consequences for affected patients. Histopathologically, angiofibromas consist of a mixture of vascular and interstitial cells. Traditional treatment options include cryotherapy, electrocoagulation, radiofrequency ablation, dermabrasion, laser treatment, and topical agents such as podophyllotoxin. However, complications such as pain, postinflammatory hyperpigmentation, scarring, and the frequent recurrence rate reduce the utility of these treatment options. The introduction of topical rapamycin marked a turning point for treatment of facial angiofibromas; however, the lack of a standardized formulation, limited insurance coverage, and significant financial cost restrict universal access for patients and their caregivers. Both oral and topical β-blockers have proven extremely effective treatments for superficial vascular tumors such as hemangiomas and pyogenic granulomas. Topical β-blockers may potentially be useful for treatment of angiofibromas considering these lesions also contain a vascular component. Here we present an exploratory case report of a patient with tuberous sclerosis complex who had significant clinical improvement of her facial angiofibromas utilizing a "split-face" comparison protocol of topical timolol 0.5% gel after full-field treatment with ablative fractional laser resurfacing and pulsed-dye laser.
血管纤维瘤是结节性硬化症最常见的皮肤表现。血管纤维瘤可能会导致容貌毁损、出血、瘙痒和红斑,并可能给受影响的患者带来严重的心理社会后果。在组织病理学上,血管纤维瘤由血管和间质细胞混合组成。传统的治疗方法包括冷冻疗法、电凝术、射频消融、磨皮术、激光治疗以及外用药物如鬼臼毒素。然而,疼痛、炎症后色素沉着、瘢痕形成等并发症以及较高的复发率降低了这些治疗方法的实用性。外用雷帕霉素的引入标志着面部血管纤维瘤治疗的一个转折点;然而,缺乏标准化配方、有限的保险覆盖范围和高昂的经济成本限制了患者及其护理人员的普遍使用。口服和外用β受体阻滞剂已被证明是治疗浅表血管肿瘤如血管瘤和化脓性肉芽肿的极其有效的方法。考虑到这些病变也包含血管成分,外用β受体阻滞剂可能对血管纤维瘤的治疗有用。在此,我们报告一例结节性硬化症患者的探索性病例,该患者在接受剥脱性分次激光换肤和脉冲染料激光全脸治疗后,采用“分脸”比较方案外用0.5%噻吗洛尔凝胶,面部血管纤维瘤有显著临床改善。