Jabbar A S
King Faisal Specialist Hospital & Research Centre, Department of Surgery, Riyadh, Kingdom of Saudi Arabia.
Eur J Surg Oncol. 2000 Sep;26(6):612-4. doi: 10.1053/ejso.2000.0957.
Perianal extramammary Paget's disease is a rare disorder of unknown aetiology and is frequently associated with malignancy. Since Sir James Paget's original description, extramammary Paget's disease has been surrounded by controversy, speculation and much interest on the part of surgeons, pathologists and dermatologists. This case report draws attention to this rare condition, which should be considered in the differential diagnosis of perianal disorders. The physician must maintain a high index of suspicion, especially in cases with characteristic lesions unresponsive to conventional dermatological therapy. The importance of awareness of the condition is stressed and the diagnosis and treatment are commented upon. Treatment and prognosis depend on the presence of an underlying invasive carcinoma. An adequate initial evaluation and long-term follow up are necessary to identify recurrence and development of other malignancies.
肛周乳腺外佩吉特病是一种病因不明的罕见疾病，常与恶性肿瘤相关。自詹姆斯·佩吉特爵士最初描述以来，乳腺外佩吉特病一直存在争议、猜测，受到外科医生、病理学家和皮肤科医生的广泛关注。本病例报告提请注意这种罕见疾病，在肛周疾病的鉴别诊断中应考虑到这一点。医生必须保持高度的怀疑指数，尤其是对于那些具有特征性病变且对传统皮肤科治疗无反应的病例。强调了认识该疾病的重要性，并对诊断和治疗进行了评论。治疗和预后取决于是否存在潜在的浸润性癌。进行充分的初始评估和长期随访对于识别复发和其他恶性肿瘤的发展是必要的。
