Tarng S J, Fan H A, Lin S E, Lai C R, Tang R P, You Y D, Chen J S, Chang-Chieh C R, Wang J Y, Lin J C
Department of Colon & Rectal Surgery, Chang Gung Memorial Hospital, Kaohsiung, Taiwan, R.O.C.
Changgeng Yi Xue Za Zhi. 1990 Dec;13(4):314-21.
Over the past nine years (from 1981 to 1989), four patients with perianal Paget's disease were treated. All were male with an average age of 58.5 years. Clinically, perianal Paget's disease manifests as a slowly enlarging eczematous, and sharply demarcated perianal skin rash that may be oozing or itching. In the characteristic pathology finding, Paget's cells appear as large, rounded signet-ring cells with abundant mucin stain positive cytoplasm in the basal layer of the acanthotic epidermis. All but one, who suffered from primary sweat gland carcinoma, had underlying rectal adenocarcinoma. The first two cases expired soon after a delayed diagnosis of terminal underlying malignancy. Only in the later two cases was there a preoperative suspicion of perianal Paget's disease. There is often a delay in diagnosis due to clinical ignorance. Patients with persisting perianal skin rash should be biopsied frequently. If perianal Paget's disease is diagnosed, the underlying malignancy should be surveyed and managed thoroughly.
在过去九年（1981年至1989年）间，对4例肛周佩吉特病患者进行了治疗。所有患者均为男性，平均年龄58.5岁。临床上，肛周佩吉特病表现为缓慢增大的湿疹样、边界清晰的肛周皮疹，可能有渗出或瘙痒。在特征性病理表现中，佩吉特细胞表现为大的、圆形的印戒细胞，在棘层肥厚的表皮基底层有丰富的黏液染色阳性细胞质。除1例患有原发性汗腺癌外，其余均有潜在的直肠腺癌。前两例在晚期潜在恶性肿瘤诊断延迟后不久死亡。仅后两例术前怀疑有肛周佩吉特病。由于临床认识不足，诊断往往会延迟。持续存在肛周皮疹的患者应经常进行活检。如果诊断为肛周佩吉特病，应全面检查并妥善处理潜在的恶性肿瘤。
