Buhle Anna C, Fagan Kiley K, Johnson Nathan M, Grider Douglas J
Section of Dermatology, Department of Internal Medicine, Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA Department of Basic Science Education, Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA.
Dermatol Online J. 2022 Oct 15;28(5). doi: 10.5070/D328559247.
Linear porokeratosis is a rare variant of porokeratosis that is characterized by unilateral lesions along the lines of Blaschko. Like all variants of porokeratosis, linear porokeratosis is characterized by the histopathologic finding of cornoid lamellae bracketing the lesion. The underlying pathophysiology involves a two-hit post-zygotic knockdown of genes involved in mevalonate biosynthesis in embryonic keratinocytes. Although there is currently no standard or effective treatment, therapies targeted to rescue this pathway and restore keratinocyte cholesterol availability are promising. Presented here is a patient with a rare, extensive case of linear porokeratosis treated with compounded 2% lovastatin/2% cholesterol cream leading to partial resolution of the plaques.
线状汗孔角化症是汗孔角化症的一种罕见变体,其特征为沿Blaschko线分布的单侧损害。与所有汗孔角化症变体一样,线状汗孔角化症的组织病理学表现为损害周围有鸡眼样板层。其潜在的病理生理学涉及胚胎角质形成细胞中参与甲羟戊酸生物合成的基因在合子后发生两次打击而功能缺失。尽管目前尚无标准或有效的治疗方法,但针对挽救该途径并恢复角质形成细胞胆固醇供应的疗法前景广阔。本文介绍了一名患有罕见广泛性线状汗孔角化症的患者,使用复方2%洛伐他汀/2%胆固醇乳膏治疗后,斑块部分消退。