外阴佩吉特病的研究进展

Progress of Paget disease of the vulva

外阴佩吉特病属于乳腺外佩吉特病最常见的类型,是一种临床罕见的发生于外阴的恶性肿瘤。目前该病具体发病机制尚不明确,病灶主要累及顶泌汗腺丰富的区域,如外生殖器。临床表现无明显异质性,可表现为病变区红斑、瘙痒、皮屑和刺痛,严重者可伴有糜烂、渗液等。确诊需要组织病理学检查和免疫组织化学。临床医生对外阴佩吉特病认识不足,患者常常被误诊为湿疹、皮炎而导致病情延误。现对外阴佩吉特病的研究进展进行综述,旨在加深临床医师对该病的认识,切实提高临床诊治能力。

Paget disease of the vulva is the most common type of extramammary Paget disease and is a rare clinical malignancy occurring in the vulva.At present,the specific pathogenesis is not clear,and the lesions mainly involve areas rich in apocrine sweat glands,such as the external genitalia.The clinical manifestations show no obvious heterogeneity,and may be characterized by erythema,pruritus,scurf and tingling in the lesion area,with erosion and exudation in severe cases.Diagnosis requires histopathological examination and immunohistochemistry.Paget disease of the vulva is often misdiagnosed as eczema or dermatitis due to the lack of knowledge about this disease in clinicians.This article reviews the current research progress of Paget disease of the vulva,in order to deepen clinicians'understanding of this disease and effectively improve clinical diagnosis and treatment abilities.