European Interbalkan Medical Center, 10 Asklipiou Street, 55535 Pylaia, Greece.
3rd Surgical Department, University General Hospital of Thessaloniki "AHEPA", School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, 1st St. Kiriakidi Street, 54621 Thessaloniki, Greece.
Medicina (Kaunas). 2021 Sep 27;57(10):1029. doi: 10.3390/medicina57101029.
Extramammary Paget's disease is a rare condition, affecting 6.5% of all patients with Paget's disease. The most common extramammary site is the vulvar area. Although diagnosis in some patients is difficult to set, early diagnosis is of high importance in order to detect the irreversible progression of the lesion early and prevent distant metastasis. An 89-year-old female and a 69-year-old female presented within three months with an eczematous lesion with leukoplakia in the vulva. The incisional biopsy of the skin revealed extramammary Paget's disease. Both patients underwent a surgical wide local excision of the lesion and the specimens were sent for histopathological examination. Extramammary Paget's disease has a high potential for distant malignancies and local recurrence, dictating that surgical excision is the most efficient treatment. The rareness of the condition and the diagnostic difficulties underline the need for early skin biopsy, which is the most efficient diagnostic tool.
派杰氏病(Paget's disease)是一种罕见的疾病,占所有派杰氏病患者的 6.5%。最常见的派杰氏病发生部位是外阴。尽管一些患者的诊断较难确定,但早期诊断非常重要,以便及早发现病变的不可逆转进展并预防远处转移。一位 89 岁女性和一位 69 岁女性在三个月内出现外阴湿疹样病变伴白色斑块。皮肤切开活检显示为派杰氏病。两名患者均接受了病变的广泛局部切除术,标本均进行了组织病理学检查。派杰氏病有发生远处恶性肿瘤和局部复发的高风险,因此手术切除是最有效的治疗方法。这种疾病非常罕见,诊断也有难度,这突出了早期皮肤活检的必要性,皮肤活检是最有效的诊断工具。