Department of Dermatology (Pav. R) and Laboratory of Dermatopathology (Bldg 10), Ed. Herriot Hospital, Lyon cx 03, France.
J Eur Acad Dermatol Venereol. 2011 Mar;25(3):296-301. doi: 10.1111/j.1468-3083.2010.03784.x.
Circumscribed palmoplantar hypokeratosis (CPH) is a recently recognized, rarely reported dermatosis that shows characteristic clinicopathological features; however, its pathogenesis remains unknown.
The aim of this study was to get further insight into the pathogenesis of CPH.
An immunohistological study was performed on five cases of CPH to investigate the expression of several epidermal proliferation and differentiation proteins, with emphasis on those involved in corneocyte desquamation [including corneodesmosin, kallikrein 5 and lympho-epithelial Kazal type inhibitor (LEKTI)].
In three of five cases, a decreased expression of LEKTI, corneodesmosin and filaggrin was found, along with an increased expression of kallikrein 5 and keratin 6. The expression of several other antigens (including involucrin, Ki67, p63, CD138/syndecan I, EGF-R) did not present a consistently different pattern as compared with the unaffected epidermis.
The immunohistopathologic features of CPH suggest that an altered (accelerated) corneocyte desquamation process could be the main pathological mechanism underlying the development of lesions.
局限性掌跖角化病（CPH）是一种新近被认识到的、罕见报道的皮肤病，具有特征性的临床病理特征；然而，其发病机制尚不清楚。
本研究旨在深入了解 CPH 的发病机制。
对 5 例 CPH 进行免疫组织化学研究，以研究几种表皮增殖和分化蛋白的表达情况，重点研究与角质形成细胞脱落有关的蛋白[包括桥粒芯糖蛋白、激肽释放酶 5 和淋巴上皮 Kazal 型抑制剂（LEKTI）]。
在 5 例中的 3 例中，发现 LEKTI、桥粒芯糖蛋白和丝聚蛋白的表达减少，而激肽释放酶 5 和角蛋白 6 的表达增加。与正常表皮相比，其他几种抗原（包括兜甲蛋白、Ki67、p63、CD138/ syndecan I、EGF-R）的表达并没有呈现出一致的不同模式。
CPH 的免疫组织病理学特征表明，异常（加速）的角质形成细胞脱落过程可能是病变发展的主要病理机制。
