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汗孔角化症

Porokeratosis

作者信息

Williams Grant M., Dreyer Mark A., Fillman Eric P.

机构信息

Keesler AFB

Intercoastal Medical Group

Abstract

Porokeratosis is an uncommon disorder of keratinization that presents with keratotic papules or annular plaques with an elevated border. The distinct histologic hallmark of porokeratosis is cornoid lamella, a column of tightly fitted parakeratotic cells in the upper epidermis. There are multiple clinical variants of porokeratosis, including: Disseminated superficial actinic porokeratosis. Classical porokeratosis of Mibelli. Porokeratosis plantaris, palmaris, et disseminate. Linear porokeratosis  . Additionally, rare variants include: Genitogluteal porokeratosis. Facial porokeratosis. Giant porokeratosis. Porokeratosis ptychotropica. Hypertrophic verrucous porokeratosis. Eruptive pruritic papular porokeratosis. Follicular porokeratosis . Reticulate porokeratosis  . Variants can occur together but rarely do. Porokeratosis is a precancerous lesion that can undergo malignant transformation. Evaluation of porokeratosis is best conducted with a biopsy of the elevated border. Although multiple therapies exist for porokeratosis (including topical, systemic, and surgical), there are no standard guidelines for treatment.

摘要

汗孔角化症是一种罕见的角化异常疾病,表现为角化性丘疹或具有隆起边缘的环状斑块。汗孔角化症独特的组织学特征是鸡眼样板层,即上表皮中一列紧密排列的不全角化细胞。汗孔角化症有多种临床变体,包括:播散性浅表性光化性汗孔角化症、经典型米贝利汗孔角化症、掌跖播散性汗孔角化症、线状汗孔角化症。此外,罕见变体包括:臀生殖器汗孔角化症、面部汗孔角化症、巨大型汗孔角化症、匐行性汗孔角化症、肥厚性疣状汗孔角化症、发疹性瘙痒性丘疹性汗孔角化症、毛囊性汗孔角化症、网状汗孔角化症。这些变体可能同时出现,但很少见。汗孔角化症是一种癌前病变,可发生恶性转化。对汗孔角化症的评估最好通过对隆起边缘进行活检来进行。虽然有多种治疗汗孔角化症的方法(包括局部、全身和手术治疗),但目前尚无标准的治疗指南。

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