Farrell A M, Charnock F M, Millard P R, Wojnarowska F
Department of Dermatology, Oxford Radcliffe Hospital, Oxford OX2 8LQ, UK.
Br J Dermatol. 1999 Jul;141(1):146-9. doi: 10.1046/j.1365-2133.1999.02937.x.
We report two women in whom vulval Paget's disease occurred in association with local adenocarcinoma and previous breast adenocarcinoma. The first patient presented at the age of 83 years with moist erythematous changes over the perineum and an indurated area near the anus. Biopsy of the indurated area showed Paget's cells throughout the epidermis and, below, adenocarcinoma infiltrating the dermis. Ten years previously, she had undergone a left mastectomy for infiltrating ductal carcinoma of the breast. The second patient was diagnosed as having Paget's disease at the age of 74 years. A vulval biopsy showed Paget's cells in the epidermis but, in addition, there were changes suggestive of adenocarcinoma of the sweat glands. Her symptoms of vulval itching had started at the age of 45 years and had led to a simple vulvectomy at the age of 57 years. Retrospective review of this vulvectomy specimen showed Paget's disease. She had also previously been treated for infiltrating ductal adenocarcinoma of the breast and adenocarcinoma of the rectum. The management of Paget's disease is difficult because of its high recurrence rate and, as illustrated by our two cases, treatment is difficult if the patients are elderly and in poor general health.
我们报告了两名女性，她们的外阴佩吉特病与局部腺癌及既往乳腺癌相关。第一名患者83岁，会阴出现湿润性红斑改变，肛门附近有硬结区。硬结区活检显示表皮全层有佩吉特细胞，其下腺癌浸润真皮。10年前，她因乳腺浸润性导管癌接受了左乳切除术。第二名患者74岁时被诊断为佩吉特病。外阴活检显示表皮有佩吉特细胞，但此外还有提示汗腺腺癌的改变。她45岁开始出现外阴瘙痒症状，57岁时接受了单纯外阴切除术。对该外阴切除标本的回顾性检查显示为佩吉特病。她此前还曾接受过乳腺浸润性导管腺癌和直肠腺癌的治疗。佩吉特病的治疗很困难，因为其复发率高，而且如我们这两个病例所示，如果患者年老且全身健康状况不佳，治疗会很困难。
