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播散性浅表性光化性汗孔角化症

Disseminated Superficial Actinic Porokeratosis

作者信息

Le Cuong, Bedocs Paul M.

机构信息

US Derm Partners

Ohio University

PMID:29083728
Abstract

Disseminated superficial actinic porokeratosis (DSAP) is a disease of disordered keratinization. This condition represents 1 of 6 porokeratosis variants and involves more extensive distribution than most others. Linear porokeratosis, porokeratosis of Mibelli, punctate porokeratosis, porokeratosis palmaris et plantaris disseminata, and disseminated superficial porokeratosis comprise the remaining 5 variants. Other rare forms include porokeratosis ptychotropica, facial porokeratosis, giant porokeratosis, hypertrophic verrucous porokeratosis, reticulate porokeratosis, and eruptive pruritic papular porokeratosis. The eruptive form has been linked to malignancy, immunosuppression, and a pro-inflammatory state. Lesions develop across the body. Risk factors for porokeratosis include genetic predisposition, immunosuppression, and ultraviolet light exposure. A defining feature of all porokeratosis variants is the cornoid lamella, which appears histologically as a column of parakeratotic cells. This structure is characterized by a raised ridge circumscribing porokeratotic lesions. DSAP lesions begin as pink to brown papules and macules with a raised border in sun-exposed areas. These lesions may be asymptomatic or mildly pruritic. DSAP is considered precancerous, with a 7.5% to 10% risk of malignant transformation to squamous cell carcinoma (SCC) or basal cell carcinoma (BCC). Multiple treatment options exist, including topical diclofenac, photodynamic therapy (PDT), 5-fluorouracil (5-FU), imiquimod, vitamin D analogs (eg, calcipotriene), retinoids, and lasers.

摘要

播散性浅表性光化性汗孔角化症(DSAP)是一种角化异常性疾病。这种病症是6种汗孔角化症变体之一,其分布比大多数其他变体更为广泛。线状汗孔角化症、米贝利汗孔角化症、点状汗孔角化症、掌跖播散性汗孔角化症和播散性浅表性汗孔角化症构成了其余5种变体。其他罕见形式包括褶叠性汗孔角化症、面部汗孔角化症、巨大型汗孔角化症、肥厚疣状汗孔角化症、网状汗孔角化症和发疹性瘙痒性丘疹性汗孔角化症。发疹型与恶性肿瘤、免疫抑制和促炎状态有关。病变遍布全身。汗孔角化症的危险因素包括遗传易感性、免疫抑制和紫外线暴露。所有汗孔角化症变体的一个决定性特征是角样板,在组织学上它表现为一列不全角化细胞。这个结构的特点是有一个隆起的边缘环绕着汗孔角化症病变。DSAP病变开始时为粉红色至棕色丘疹和斑疹,在暴露于阳光的部位有隆起的边缘。这些病变可能无症状或有轻度瘙痒。DSAP被认为是癌前病变,有7.5%至10%的恶变风险转化为鳞状细胞癌(SCC)或基底细胞癌(BCC)。有多种治疗选择,包括外用双氯芬酸、光动力疗法(PDT)、5-氟尿嘧啶(5-FU)、咪喹莫特、维生素D类似物(如卡泊三醇)、维甲酸和激光。

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