乳房外Paget病1例

患者男，72岁。因外阴部红斑、渗出伴瘙痒4年，于2005年5月6日就诊。患者4年前无明显诱因阴茎根部出现红斑伴瘙痒，皮损逐渐向邻近皮肤蔓延，时有渗液及脓性分泌物，曾多次在外院就诊，均诊断为湿疹，给予曲安奈德益康唑乳膏等药物治疗，皮损时好时坏，范围逐渐扩大，病程中无发热、消瘦、乏力等。既往有痛眦病史20年，高血压病史1年余。体格检查：系统俭查未见异常。[第一段]     

乳房外Paget病1例

患者男，6l岁。阴囊左侧皮肤瘙痒性皮疹2年，于2002年8月2日就诊。患者于2年前阴囊部位起小丘疹，伴瘙痒，皮疹逐渐增多、增厚，日寸有渗出，一直不消退。发病后，曾至多家医院就诊，均诊断为“湿疹”，给予抗组胺药及皮质类固醇软膏外用，疗效不明显。患者既往身体健康，否认有糖尿病病史。体格检查：局部淋巴结不肿大，心肺等无异常发现。皮肤科检查。     

乳房外Paget病1例

患者男,73岁。阴茎根部、阴囊出现红斑伴瘙痒13年,糜烂、渗出、溃疡、出现肿物伴异味1年。组织病理示局部区域有表皮下浸润,PAS(+),Alcianblue(-),免疫组化CEA(3+),CK(2+),HMB45(-),S-100(-)。诊断:乳房外Paget病。     

乳房外Paget病1例

患者男,58岁。左腹股沟反复起红斑,糜烂伴瘙痒8年,于2004年2月来我院就诊。患者8年前无明显诱因于左侧腹股沟出现红斑伴瘙痒,曾多次就医,以“湿疹”给予糖皮质激素类软膏外用,皮损可减轻,但易反复;随后皮损逐渐扩大,伴糜烂及渗出,俚未出现畏寒,发热等全身症状。     

乳房外Paget病1例

患者男 ,64岁。阴囊皮疹偶伴渗出、瘙痒 10年。病理检查示 :全层表皮均见Paget细胞 ,有的成巢状 ,个别真皮乳头可见基底突破。诊断为 :乳房外Paget病 ,突破基底膜。     

乳房外Paget病1例

患者女,73岁.外阴及会阴红色斑片、糜烂3年伴疼痛1年,于2011年7月18日就诊.患者3年前无明显诱因外阴和阴唇部出现红斑,无糜烂,无自觉症状,未经治疗.皮损逐渐向会阴部发展,并且小阴唇红斑上出现点状糜烂伴少许渗液.曾在当地妇产科就诊,具体诊断与用药不详,未见好转.近1年患者自觉皮损持续性隐痛,且肛周出现红斑,无尿频、尿痛、无腹痛、腹泻,无发热.自起病以来患者精神、饮食、睡眠较差.2001年8月15日曾在当地医院行直肠腺癌手术治疗.     

乳房外Paget病1例

患者男,81岁。会阴部反复起红斑、糜烂伴瘙痒12年,加重3天。组织病理示表皮内可见弥漫呈巢状或散在分布的Paget细胞,细胞大,胞质丰富而淡染,呈空泡状,核大深染,核周有空晕。诊断为乳房外Paget病。     

乳房外Paget病1例

患者男,63岁.因阴茎根部左侧出现红斑、糜烂,伴瘙痒7年,于2011年7月9日来监利县人民医院科就诊.患者7年前无明显诱因阴茎根部出现红斑,伴轻度瘙痒,无疼痛.到当地医院多次就诊,先后按皮炎、湿疹、真菌感染治疗(具体不详),自觉病情偶有好转,瘙痒症状减轻.     

乳房外Paget病1例

患者男,54岁.因阴囊右侧皮肤红斑、糜烂、渗液13个月于2011年10月14日就诊.13个月前无明显诱因患者阴囊右侧皮肤出现直径约1 cm大红斑,轻度瘙痒,搔抓后出现糜烂、渗液.曾在当地多家医院诊为"湿疹",给予多种药膏外用及抗组胺药物口服治疗(具体不详),病情无明显好转,近4个月来自觉皮疹明显增大,患者因惧怕恶变遂来我院诊治.既往体健,无其他肿瘤病史,家族中未见类似疾病患者.     

乳房外Paget病1例

<正>1病历摘要患者男,79岁。因阴囊及阴茎丘疹和糜烂伴瘙痒10余年,加重1个月,于2016年5月30日来我科就诊。患者10余年不明原因阴囊出现芝麻大淡红色丘疹,自觉轻微瘙痒,未予诊治。皮损逐渐增多,部分融合成片,瘙痒剧烈,搔抓后形成糜烂面伴渗液,曾多次在外院就诊,均诊断为阴囊湿疹,予静脉滴注复方甘草酸苷,肌内注射卡介菌素多糖核酸,口服左西替利嗪,外用卤米松、     

乳房外Paget病

患者,男,67岁主诉：右侧阴囊斑片、结痂,伴有轻度瘙痒6年现病史：患者6年前无明显诱因右侧阴囊出现蚕豆大小红斑,表面潮湿、脱屑,微痒。多次就诊于当地医院,查真菌均阴性,拟诊“阴囊湿疹”、“体癣”等予口服抗组胺药、反复外用曲安奈德尿素乳膏、地奈德软膏、糠酸莫米松乳膏等治疗,无明显效果。皮损逐渐扩大,自觉症状不明显。     

Extramammary Paget Disease: Minimal Surgical Therapy

Background

Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm affecting apocrine gland-bearing skin which usually occurs in the anogenital area of patients older than 50 years. Although Mohs micrographic surgery (MMS) is recommended for the treatment of EMPD, wide local excision has also been performed by many other surgeons including dermatosurgeons. However, the extent of an adequate resection margin is still under debate.

Objective

The efficacy of minimal surgical therapy consisting of a wide excision combined with preoperative multiple scouting biopsies and postoperative topical imiquimod was investigated for the treatment of EMPD in Korean patients.

Methods

Between 2006 and 2012, 10 patients with primary EMPD were treated with wide surgical excision, with a surgical margin of less than 2.5 cm. Multiple preoperative scouting biopsies and postoperative topical imiquimod were also performed to delineate the lesional boundaries and to reduce the recurrence rate.

Results

During the 6-year follow-up period, complications and recurrences were not observed.

Conclusion

Minimal surgical therapy may be an effective alternative when MMS is unavailable.     

误诊6年的乳房外Paget病2例报道

乳房外Paget病(EMPD)是一种罕见的、发生于乳房外的一种表皮内存在Paget细胞的湿疹样癌。可发生于外阴、阴囊、腹股沟、阴茎、肛周等大汗腺丰富的部位,常常与表皮内肿瘤和内脏肿瘤有关。由于表现多种多样易出现误诊。在病理上应与Bowen病、浅表型恶性黑色素瘤区别,发生于外阴的乳房外Paget病在临床上更易误诊为湿疹、脂溢性皮炎。本文介绍了2例被误诊的Paget病,同时复习了国内外有关本病的一些诊断和治疗方面的进展。     

Extramammary Paget disease

We report the case of a 60-year-old man with penile-scrotal extramammary Paget disease (EMPD). The patient initially underwent Mohs micrographic surgery, but the margins remained positive after several sections; multiple scouting punch biopsies used to define the extent of the tumor were also positive. Because of concerns about functional impairment and cosmesis associated with wide local excision, the patient instead chose treatment with topical 5 percent imiquimod cream as a cytoreductive and margin-defining treatment. Owing to the association between EMPD and underlying malignant conditions, a thorough metastatic evaluation is necessary, particularly to rule out genitourinary cancer in the setting of penile-scrotal EMPD. Management of EMPD is complicated by the multifocal, non-contiguous nature of the disease and the presence of clinically occult extensions. As a result, recurrence rates after surgery are high. Several non-surgical modalities have been used to treat EMPD, which include radiotherapy, topical imiquimod, topical 5-fluorouracil, topical bleomycin, photodynamic therapy, CO2 laser ablation, and topical retinoids. Systemic chemotherapy also has been used to treat advanced EMPD. However, because EMPD is so uncommon, clinical trials comparing the various methods of treatment are lacking. Regardless of the mode of treatment, long-term follow up is essential, given the high rate of recurrence.     

乳房外Paget病并发尖锐湿疣

报告1例乳房外Paget病(EMPD)并发尖锐湿疣(CA)。患者男,72岁。阴茎、阴囊浸润性红斑伴瘙痒4年,1个月前阴茎、阴囊、龟头及包皮出现多个疣状赘生物。皮损组织病理检查:(阴茎根部皮损)表皮棘层肥厚,表皮突延长,表皮内有不等量的Paget细胞,呈圆形或卵圆形,无细胞间桥,胞质丰富,淡染,细胞核较大,伴慢性炎症;(阴茎赘生物)鳞状上皮呈乳头样增生伴挖空样细胞。免疫组化示:CK7(+)、CerbB2(+)、CK5/6(+)、P53(+)、Ki-67约20%(+)、CK20(-)、s-100蛋白(-)及HMB45(-)。诊断:EMPD并发CA。     

Perianal and Vulvar Extramammary Paget Disease: A Report of Six Cases and Mapping Biopsy of the Anal Canal

Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.     

发生多处转移的乳房外Paget病1例

患者男,67岁。阴囊和右侧腹股沟出现红斑和丘疹,伴瘙痒7年,多次复发,予手术切除。皮损组织病理示:表皮全层见胞浆透亮的Paget细胞浸润。CT,ECT证实肝、肺、骨多处转移。诊断:乳房外Paget病(多处转移)。