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汗孔角化症的临床流行病学研究

A Clinico-Epidemiological Study on Porokeratosis.

作者信息

Shirahatti Trishala, Bangaru H, Sathish S

机构信息

From the Department of Dermatology, St Johns Medical College Hospital, Bengaluru, Karnataka, India.

Department of Dermatology, Mysore Medical College and Research Institute, Mysore, Karnataka, India.

出版信息

Indian J Dermatol. 2024 Jul-Aug;69(4):365. doi: 10.4103/ijd.ijd_131_24. Epub 2024 Aug 19.

DOI:10.4103/ijd.ijd_131_24
PMID:39296684
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11407577/
Abstract

Porokeratosis (PK) is a chronic progressive disorder of keratinization characterized clinically by hyperkeratotic papules or plaques surrounded by a threadlike, elevated border that expands centrifugally. Pathogenesis involves heterozygous mutations in mevalonate kinase enzyme. The most common variants are disseminated superficial actinic porokeratosis (DSAP) and PK of Mibelli. All forms show a thin column of parakeratosis, the cornoid lamella, representing the active border. Dermoscopy reveals central brownish discoloration surrounded by a single hypopigmented band and a peripheral 'white track'. Long-standing cases of PK may undergo malignant transformation. UV-protection and topical agents, such as 5-fluorouracil, imiquimod, calcipotriol, tretinoin and oral retinoids are helpful. To study the clinical and epidemiological pattern of PK and the clinico-dermoscopic and histopathological correlation of PK. A prospective cross-sectional study was conducted on the patients attending the dermatology outpatient department (OPD) over 9 months with suspected features of PK. These patients were evaluated clinically and subjected to dermoscopy and histopathology. Statistical Package for the Social Sciences (SPSS) Of 11 patients, there were four (36.36%) males and seven (63.66%) females. The youngest was 18-year-old boy, and the eldest was 63-year-old man. The clinically most common type was PK of Mibelli with five (45.45%) cases. The most common dermoscopic feature observed was double-marginated, white peripheral border. The most common histopathological feature noted was cornoid lamella. PK is a rare skin disorder with a wide spectrum of clinical variants. Dermoscopy and histopathology aid in diagnosis, whereas dermoscopy plays a pivotal role in early and non-invasive diagnosis. Regular follow-up is mandatory to watch for the development of malignancies in a few variants.

摘要

汗孔角化症(PK)是一种慢性进行性角化异常性疾病,临床特征为角化过度性丘疹或斑块,周围有丝状、隆起的边界,并呈离心性扩展。其发病机制涉及甲羟戊酸激酶酶的杂合突变。最常见的类型是播散性浅表性光化性汗孔角化症(DSAP)和米贝利汗孔角化症。所有类型均表现为一层薄的不全角化柱,即角样板,代表活跃边界。皮肤镜检查显示中央褐色色素沉着,周围有一条单一的色素减退带和一条外周“白色轨迹”。长期存在的汗孔角化症病例可能会发生恶变。紫外线防护以及局部用药,如5-氟尿嘧啶、咪喹莫特、卡泊三醇、维甲酸和口服维甲酸类药物是有帮助的。为研究汗孔角化症的临床和流行病学模式以及汗孔角化症的临床皮肤镜和组织病理学相关性。对皮肤科门诊9个月内疑似汗孔角化症特征的患者进行了一项前瞻性横断面研究。这些患者接受了临床评估,并进行了皮肤镜检查和组织病理学检查。使用社会科学统计软件包(SPSS)。11例患者中,男性4例(36.36%),女性7例(63.66%)。最年轻的是一名18岁男孩,最年长的是一名63岁男性。临床上最常见的类型是米贝利汗孔角化症,有5例(45.45%)。观察到的最常见皮肤镜特征是双边缘白色外周边界。最常见的组织病理学特征是角样板。汗孔角化症是一种罕见的皮肤疾病,有广泛的临床变异型。皮肤镜检查和组织病理学有助于诊断,而皮肤镜检查在早期和非侵入性诊断中起关键作用。对于少数变异型,定期随访以观察恶性肿瘤的发生是必要的。

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