Bipedicled scrotal flaps combined with keystone-design perforator island flaps in repairing postoperative large-area defects in 6 male patients with extramammary Paget′s disease of the perineum / 中华皮肤科杂志

Objective:To evaluate clinical efficacy of bipedicled scrotal flaps combined with keystone-design perforator island flaps in repairing postoperative large-area defects in male patients with extramammary Paget′s disease of the perineum.Methods:Clinical data were collected from 6 male patients with extramammary Paget′s disease of the perineum in Dermatology Hospital of Jiangxi Province from February 2018 to March 2019, and analyzed retrospectively. These patients were aged from 70 to 84 years (median, 77.5 years) , skin lesions involved the mons pubis, penis and scrotum, and the area of postoperative skin defects varied from 18 to 133 cm 2 (median, 96 cm 2) in size. In all the patients, mons pubis defects after tumor resection were repaired with abdominal keystone-design perforator island flaps, and scrotal and penile defects were repaired with bipedicled scrotal flaps using the remaining scrotal tissues. These patients were followed up at 1 and 3 months after surgery and every 3 months thereafter. Results:All the 6 patients were followed up for 3 - 36 months, with an average of 10 months. All flaps survived with a good color and texture match, and favorable function and appearance were achieved in both the donor and recipient sites.Conclusions:The bipedicled scrotal flaps combined with keystone-design perforator island flaps can repair postoperative large-area defects in male patients with extramammary Paget′s disease of the perineum. Moreover, the operation is simple, and good blood supply can be achieved.

Reflectance confocal microscopy-assisted surgical treatment for two cases of extramammary Paget′s disease of the vulva / 中华皮肤科杂志

In order to develop a method to more accurately determine the surgical boundary of extramammary Paget′s disease, reflectance confocal microscopy was used to determine the tumor boundary followed by modified extended excision in 2 cases of pathologically diagnosed extramammary Paget′s disease of the vulva. No residual tumor was observed in the resection margins by postoperative pathological examination at 4 positions (12, 3, 6 and 9 o′clock) , and follow-up showed no recurrence 1 year later.

Analysis of high-frequency color Doppler ultrasound characteristics of extramammary Paget′s disease / 中华皮肤科杂志

Objective:To explore high-frequency color Doppler ultrasound characteristics of extramammary Paget′s disease (EMPD) .Methods:From January 2015 to October 2019, 32 patients with pathologically confirmed EMPD were collected from the Affiliated Hospital of Jining Medical University, and characteristics of their high-frequency color Doppler ultrasound images were retrospectively analyzed.Results:Of the 32 lesions, 25 were located on the external genitalia (21 on the scrotum, 4 on the perineum) , 4 on the medial thigh, 1 on the lower abdominal wall, 1 in the perianal region, and 1 on the neck. Ipsilateral inguinal lymph node metastasis occurred in 3 patients, and bilateral inguinal lymph node metastasis in 1 patient. High-frequency ultrasonography showed no obvious abnormal ultrasound manifestations in 6 cases, and thickened skin lesions in 26 cases. According to the morphological and high-frequency ultrasound manifestations, 26 skin lesions were classified into 2 types: 21 diffuse-type lesions with unclear borders and irregular shapes, and 5 mass-type lesions with clear borders and irregular shapes. Solid and homogeneously hypoechoic areas were observed in 18 cases, and solid and heterogeneously hypoechoic areas in 8. According to the Adler blood flow classification, the 26 lesions were classified into 4 grades: 2 of grade 0, 5 of grade 1, 8 of grade 2, and 11 of grade 3.Conclusion:EMPD shows characteristic high-frequency color Doppler ultrasound manifestations, which can facilitate its clinical diagnosis.

Value of dermoscopy in differential diagnosis of extramammary Paget′s disease and chronic eczema of the vulva / 中华皮肤科杂志

Objective@#To describe the dermoscopic features of extramammary Paget′s disease (EMPD) and chronic eczema of the vulva, and to explore the value of dermoscopy in the diagnosis and differential diagnosis of the above diseases.@*Methods@#Dermoscopic images were collected from 20 patients with histopathologically confirmed vulvar EMPD and 16 patients with clinically confirmed chronic eczema of the vulva in Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Pekin Union Medical College from January 2017 to April 2018, and retrospectively analyzed. Fisher′s exact test was used to compare the prevalence of dermoscopic features between the two groups.@*Results@#As dermoscopy showed, the milky red background was observed in 19 EMPD patients and in only 1 patient with chronic eczema, and there was a significant difference in the prevalence of milky red background between the two groups (P < 0.001) . White scales were the most common desquamation, and were observed in 9 EMPD patients and 13 patients with chronic eczema (P = 0.128) . Blood vessels were uniformly distributed in EMPD patients, including dotted-globular vessels in 19 and linear vessels in 14, while blood vessels were distributed in a patchy or clustered pattern in the patients with chronic eczema, including dotted-globular vessels in 16 and linear vessels in 7. There was a significant difference in the prevalence of different distribution patterns of blood vessels between the two groups (P < 0.001) . Bright white streaks, bright white structureless area and reticular structure were observed in 19, 20 and 19 EMPD patients respectively, and in 1, 1 and 1 patient with chronic eczema respectively, and there were significant differences in the prevalence of the above 3 structures between the two diseases (all P < 0.001) .@*Conclusion@#Vulvar EMPD and chronic eczema both show characteristic dermoscopic features, and dermoscopy is of great value to the diagnosis and differential diagnosis of EMPD.

Ultrasonographic characteristics of extramammary Paget's disease / 中国医学影像技术

Objective To explore the ultrasonographic features of extramammary Paget's disease (EMPD). Methods The ultrasonographic data of 41 patients with EMPD confirmed by pathology were retrospectively analyzed, and the ultrasonographic features of EMPD were observed. Results Among the 41 patients of EMPD, lesions located at vulva in 33 cases, at groin in 2, at perianal in 2 cases, while at upper chest wall, lower abdominal wall, forehead and thumb in 1 case, respectively. Among 36 patients with ultrasonographic found lesions, EMPD could be divided into diffuse type and mass type according to the morphology of the lesions. There were 31 diffuse type lesions with irregular shape and unclear margin. Ultrasound showed skin thickening in 30 lesions and no thickening in 1, hypoechoic echo in 8 and slightly hypoechoic echo in 23, while CDFI blood flow demonstrated grade 0 in 2, grade 1 in 6, grade 2 in 11, grade 3 in 12. Inguinal abnormal lymph nodes were found in 9 patients with diffuse type EMPD. There were 5 patients of mass type EMPD with clear margins, among them ultrasound showed 1 patient with skin thickening and 4 without thickening, and hypoechoic lesions were found in 3 patients and slightly hypoechoic lesions in 2 patients. The shape of 4 lesions were regular and 1 was irregular, 1 lesion with CDFI blood flow of grade 0, 2 of grade 1, 1 of grade 2, 1 of grade 3. The abnormal inguinal lymph nodes were found in 3 patients. Ultrasonography showed no obvious abnormality in the other 5 patients (5/41, 12.20%). Conclusion Ultrasound could sensitively identify abnormal echo and blood flow in EMPD lesions. Clinical manifestations should be considered in ultrasound diagnosis of EMPD.

Value of dermoscopy in differential diagnosis of extramammary Paget's disease and chronic eczema of the vulva / 中华皮肤科杂志

Objective To describe the dermoscopic features of extramammary Paget's disease (EMPD) and chronic eczema of the vulva,and to explore the value of dermoscopy in the diagnosis and differential diagnosis of the above diseases.Methods Dermoscopic images were collected from 20 patients with histopathologically confirmed vulvar EMPD and 16 patients with clinically confirmed chronic eczema of the vulva in Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Pekin Union Medical College from January 2017 to April 2018,and retrospectively analyzed.Fisher's exact test was used to compare the prevalence of dermoscopic features between the two groups.Results As dermoscopy showed,the milky red background was observed in 19 EMPD patients and in only 1 patient with chronic eczema,and there was a significant difference in the prevalence of milky red background between the two groups (P ＜0.001).White scales were the most common desquamation,and were observed in 9 EMPD patients and 13 patients with chronic eczema (P =0.128).Blood vessels were uniformly distributed in EMPD patients,including dotted-globular vessels in 19 and linear vessels in 14,while blood vessels were distributed in a patchy or clustered pattern in the patients with chronic eczema,including dotted-globular vessels in 16 and linear vessels in 7.There was a significant difference in the prevalence of different distribution patterns of blood vessels between the two groups (P ＜ 0.001).Bright white streaks,bright white structureless area and reticular structure were observed in 19,20 and 19 EMPD patients respectively,and in 1,1 and 1 patient with chronic eczema respectively,and there were significant differences in the prevalence of the above 3 structures between the two diseases (all P ＜ 0.001).Conclusion Vulvar EMPD and chronic eczema both show characteristic dermoscopic features,and dermoscopy is of great value to the diagnosis and differential diagnosis of EMPD.

Uso do imiquimode para tratamento da doença de Paget extramamária: série de quatro casos / Imiquimod use for the treatment of extramammary Paget disease: series of four cases

Introdução: A doença de Paget extramamária é constituída por adenocarcinoma da pele em áreas de glândulas apócrinas. Trata-se de neoplasia rara cujo tratamento cirúrgico aparenta resultados satisfatórios. Devido às altas taxas de recorrência, entretanto, as cirurgias são potencialmente mutilantes. O imiquimode é imunoestimulador tópico utilizado no tratamento de verrugas anogenitais e carcinomas in situ. Seu uso tem sido descrito na literatura científica para terapia da doença de Paget extramamária com resultados satisfatórios. Objetivo: Descrever a evolução de quatro casos de doença de Paget extramamária tratados com imiquimode, para avaliação da evolução e resposta terapêutica. Métodos: Foi realizado estudo retrospectivo em serviço de dermatologia da cidade de São Paulo com revisão de prontuários de todos os pacientes com diagnóstico de doença de Paget extramamária e tratados com imiquimode de janeiro de 2011 a julho de 2018. Resultados: Foram incluídos quatro pacientes, três com lesão vulvar e um com lesão em bolsa escrotal. Duas mulheres evoluíram com resolução total da doença, uma não apresentou alteração, e o homem evolui com regressão de 70% da lesão, sendo submetido à exérese cirúrgica de área consideravelmente menor do que a da lesão inicial. Conclusões: O imiquimode se apresenta como método terapêutico válido no tratamento da doença de Paget extramamária.

Introduction: Extramammary Paget disease consists of a cutaneous adenocarcinoma in areas of apocrine glands. It is a rare neoplasia, and its surgical treatment yields satisfactory results. However, due to the high recurrence rates, surgeries are potentially disfiguring. Imiquimod is a topical immune-stimulant used for the treatment of anogenital warts and in situ carcinomas. Its use has been described in the scientific literature for the treatment of Extramammary Paget disease with satisfactory results. Objective: Describe the course of four cases of Extramammary Paget disease treated with imiquimod, to evaluate evolution and therapeutic response. Methods: A retrospective study was performed in a service of dermatology in the city of São Paulo, with the review of medical records of all patients diagnosed with Extramammary Paget disease and treated with imiquimod from January 2011 to July 2018. Results: Four patients were included, three with vulvar lesion and one with scrotal lesion. Two women presented completed resolution of the disease, one did not respond and the man progressed with 70% regression of the lesion, having undergone surgical excision of a considerably smaller area than the initial lesion. Conclusions: Imiquimod presents as a valid therapeutic modality for the treatment of Extramammary Paget disease.

Effect of surgery combined with aminolevulinic acid-based photodynamic therapy on the recurrence of extramammary Paget's disease / 中华皮肤科杂志

Objective To evaluate the effect of surgery combined with aminolevulinic acid (ALA)-based photodynamic therapy (PDT) on the prognosis of extramammary Paget's disease.Methods A prospective open-labelled controlled trial was conducted.A total of 38 patients with pathologically comfirmed extramammary Paget's disease were enrolled from Shanghai Dermatology Hospital,and divided into 2 groups to be treated with surgery alone (surgery alone group,n =21) or surgery combined with ALA-PDT (combination group,n =17).Patients in the combination group received ALA-PDT after the surgery once every two weeks for 3 sessions.All the patients were followed up once every three months for more than 12 months,and the incidence of relapse was evaluated and compared between the 2 groups after the treatment.Results During the follow-up of 12-58 months (mean,35.45 ± 16.98 months),7 patients in the surgery alone group experienced relapse,and the median time to relapse was 9 months,with an upper quartile of 18 months and a lower quartile of 6 months.However,relapse only occurred in 1 patient in the combination group,and the time to relapse was 18 months after the end of treatment.The recurrence rate was significantly lower in the combination group than in the surgery alone group (P ＜ 0.05),and the time to relapse was also longer in the combination group than in the surgery alone group.Furthermore,ALA-PDT after the surgery was well tolerated in all the patients.Conclusion Surgery combined with ALA-PDT can reduce the recurrence rate of extramammary Paget's disease,and improve its prognosis.

Primary Cutaneous Mucinous Carcinoma with Extramammary Paget’s Disease: Eccrine or Apocrine?

Primary cutaneous mucinous carcinoma (PCMC) is an uncommon tumor of the sweat gland origin. The occurrence of PCMC is mostly in middle-aged and older patients, with a slight male predominance. Most cases of PCMC arise on the head, with a preference for eyelids. The histogenesis of PCMC, whether eccrine or apocrine, remains controversial. We report a rare case of PCMC with secondary extramammary Paget’s disease in the groin of a 75-year-old man, which favored an apocrine origin. Furthermore, based on a review of the literature, we provide several histologic clues that can be used to differentiate PCMC from metastatic mucinous carcinoma.

Analysis of clinical characters of female vulvar Paget disease / 中华妇产科杂志

Objective@#To investigate the clinical and pathological features, diagnosis and treatment of primary vulvar Paget disease (VPD) , and analyze the related factors that may affect the recurrence.@*Methods@#A retrospective study was carried out on 36 patients diagnosed as VPD pathologically from January 1983 to December 2017 at Peking Union Medical College Hospital, Chinese Academy of Medical Sciences. The clinical and pathological features, diagnosis, treatment and prognosis and the factors influencing recurrence rate of VPD were analyzed.@*Results@#(1) Totally 94% (34/36) of VPD occurred in postmenopausal women. Pruritus was counted 86% (31/36) of the main complaint. Lesions of vulvar were main symptom which had no specificity, acting as ulcer (67%, 24/36) , erythema (50%, 18/36) , depigmentation (42%, 15/36) , sclerosis (31%, 11/36) , and pigmentation (17%, 6/36) . The lesions invaded labium majus (97%, 35/36) , sometimes labium minus (53%, 19/36) , clitoris (28%, 10/36) , perianal (25%, 9/36) , orificium vaginae (3%, 1/36) , and meatus urinarius (3%, 1/36) . Approximately 19% (7/36) of VPD coexisted with intraepithelial neoplasia or adenocarcinoma of vulvar or other part of body. (2) Diagnosis and treatment: diagnosis was confirmed histologically by biopsy or pathologies after surgery, and immunohistochemical results were helpful for differential diagnosis. Surgery was the mean treatment method, 34 of all the 36 patients (94%, 34/36) underwent surgery for at least once, while 2 patients (6%, 2/36) were performed non-operative treatment. The surgical treatment included excision of focus, wide local excision, simple vulvectomy, and extensive vulvectomy. The non-operative treatment included radiotherapy, chemotherapy, laser, photodynamic therapy, and so on. (3) Prognosis: among 36 VPD patients, 4 were lost to follow-up with a 89% (32/36) follow-up rate. Median follow-up was 35.3 months (range,1 month to 31 years) . During the follow-up period, 2 patients were unable to judge whether they will relapse for the follow-up time did not reach half a year, 8 cases were unsuccessful operation, 20 cases succeeded, the achievement ratio was 71% (20/28) . Nine of twenty cases relapsed, the recurrence rate was 45% (9/20) . The median recurrence time was 14 months after operation. One patient of the 32 followed-up patients died, the mortality rate was 3% (1/32) . (4) The related factors affected the recurrence of VPD: t test was applied to the analysis of patients′ age, rank test was used in the statistics of the time of confirmed diagnosis, the length and thickness of the resection focus. Fisher test was used to calculate whether the focus were limited to the epidermis, type of surgical procedures, distance between the margin and the focus, whether tumor cells infiltrated the margin. The results showed that none of the above terms in the first operation had significant contribution to recurrence (all P>0.05) .@*Conclusions@#VPD may be a low potential malignancy, which could slowly progress into deep invasive disease. VPD is often associated with intraepithelial neoplasia or primary tumors of the vulva or somewhere else. Operations is the first choice for VPD, but consider for its high recurrence rate after operation, close follow-up should be strongly suggested.

Comparative analysis of reflectance confocal microscopy features between Paget's disease and eczema on the scrotum / 中华皮肤科杂志

Objective To analyze and compare reflectance confocal microscopy (RCM) features between Paget's disease and eczema on the scrotum.Methods RCM was performed to image scrotal lesions of 5 male patients with suspected Paget's disease and 5 male patients with eczema.Then,the scrotal lesions were resected and subjected to histopathological examination,and a comparison was conducted between the confocal microscopic and pathological findings.Results RCM imaging for the 5 cases of Paget's disease showed disorganized epidermal structure,absence of normal honeycomb structures,single or clustered pagetoid cells in the prickle cell layer.RCM imaging for the 5 cases of eczema showed intercellular edema in the prickle cell layer,reticular degeneration of blisters in all the cases,and pustule formation in some cases.Conclusion RCM features are obviously different between Paget's disease of the scrotum and scrotal eczema,so RCM imaging can facilitate the early diagnosis of Paget's disease.

Primary penoscrotal extra-mammary Paget’s disease:analysis of 22 cases / 北京大学学报(医学版)

Objective:To investigate the clinical characteristics,treatment and prognosis of primary pe-noscrotal extra-mammary Paget’s disease (PSPD).Methods:The clinical and pathological data of 22 cases of primary PSPD were retrospectively reviewed.Survival rate of the overall patients,the invasive patients,and the patients with positive surgical margin or negative surgical margin were analyzed with Ka-plan-Meier survival curve method.Results:Among all the patients with primary PSPD,the median age of onset was 64.5 (39 -84)years,the median time of disease duration was 40 (2 -300)months,and the median long diameter of lesion was 4.75 (1 -10)cm.In the study,12 patients (54.5%)were in pathological stage A1,6 patients (27.3%)were in pathological stage A2,and 4 patients (18.2%) were in pathological stage B.Scrotum and penile of most patients (n =12,54.5%)were involved,5 patients (22.7%)were scrotum only,and 5 patients (22.7%)were penile only.Erythema (n =18, 81.8%),itchiness (n =16,72.7%),ulcerate (n =12,54.5%),exudation (n =11,50.0%),and pain (n =4,18.2%)were the major manifestations.All the patients with primary PSPD were treated with wide surgical excision.The rate of invasive patients was 77.3% (n =17).Of them,6 patients had positive surgical margin.The surgical margins of non-invasive patients were all negative.Twelve patients exhibited local recurrence or/and metastases,and the status of surgical margins (P =0.015)and the depth of invasion (P =0.010)were important risk factors.Inguinal lymph nodes were generally in-volved.The difference of the delay of diagnosis between metastasis and non-metastasis was significant (P =0.040).The 5-year survival rates of the overall and invasive patients were 33.7% and 27.9%, respectively.The patients with positive surgical margin had poor prognosis.Conclusion:Primary PSPD, with the characteristics of long duration,high invasive tendency and high incidence of local recurrence or metastases,generally occurs in the elderly.Surgery should be performed at first.The delay of diagnosis, positive surgical margins and the involvement of inguinal lymph node are important risk factors.Biopsy, frozen section and inguinal lymph node biopsy (ILNB)can standardize diagnosis and treatment.

Peripheral Mohs micrographic surgery for the treatment of extramammary Paget′s disease Wan Miaojian, Ma / 中华皮肤科杂志

Objective To evaluate effectiveness of peripheral Mohs micrographic surgery for the treatment of extramammary Paget′s disease (EMPD). Methods A total of 28 patients with EMPD were treated with peripheral Mohs micrographic surgery. The depth and extent of tumor infiltration were evaluated before the surgery. One day before the surgery, 20% aminolevulinic acid hydrochloride was topically applied to determine and label surgical margins under a Wood′s lamp. After fluorescence-based localization, peritumoral skin tissues were resected and underwent frozen-section examination according to the protocol for Mohs micrographic surgery. Meanwhile, the tumor was resected. After surgery, patients were followed up every 3 - 6 months to detect local recurrence and metastasis. Results Of the 28 patients, 25 were male and 3 were female. Six patients each underwent 3 sessions of frozen-section examination, and 12 patients each received 2 sessions, with an average of 1.86 sessions for each patient. During the follow-up for 5 - 72 months, local recurrence occurred in 3 cases, and 1 patient died of tumor metastasis and uremia after 2 years of follow-up. Conclusion Peripheral Mohs micrographic surgery is a time-saving and effective treatment for EMPD.

Rare Case of Anal Canal Signet Ring Cell Carcinoma Associated with Perianal and Vulvar Pagetoid Spread

A 61-year-old woman was referred to surgery for incidentally found colonic polyps during a health examination. Physical examination revealed widespread eczematous skin lesion without pruritus in the perianal and vulvar area. Abdominopelvic computed tomography showed an approximately 4-cm-sized, soft tissue lesion in the right perianal area. Inguinal lymph node dissection and Mils' operation extended to perianal and perivulvar skin was performed. Histologically, the anal canal lesion was composed of mucin-containing signet ring cells, which were similar to those found in Pagetoid skin lesions. It was diagnosed as an anal canal signet ring cell carcinoma (SRCC) with perianal and vulvar Pagetoid spread and bilateral inguinal lymph node metastasis. Anal canal SRCC is rare, and the current case is the third reported case in the English literature. Seven additional cases were retrieved from the world literature. Here, we describe this rare case of anal canal SRCC with perianal Pagetoid spread and provide a literature review.

Mammary and extramammary Paget's disease

Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

Unilateral Pigmented Extramammary Paget's Disease of the Axilla Associated with a Benign Mole: A Case Study and a Review of Literature

Pigmented extramammary Paget's disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget's disease affecting skin that is rich in apocrine sweat glands such as the axilla, perianal region and vulva. It most commonly occurs in postmenopausal women and presents as a superficial pigmented scaly macule, mimicking a melanocytic lesion. The histological presentation is adenocarcinoma in situ with an increased number of melanocytes scattered between the Paget's cells. Therefore, PEMPD may be misdiagnosed as a melanocytic tumour both clinically and histologically. The tumour cells are usually positive for cytokeratin 7, epithelial membrane antigen, Cam 5.2, HER2, and mucicarmine stain while S100 and human melanoma black-45 highlight the processes of reactive dendritic cells. The association between Paget's cells and intratumoural reactive melanocytes is still unclear. We report our first case of PEMPD associated with an intradermal naevus involving the axilla in a 63-year-old woman.

The Effectiveness of Mapping Biopsy in Patients with Extramammary Paget's Disease

BACKGROUND: Extramammary Paget's disease (EMPD) is an intraepithelial carcinoma usually occurring on the skin or mucosa of the perineum. Clinically, it resembles eczema or dermatitis, and misdiagnosis and treatment delays are common. The treatment of choice for EMPD is a wide excision with adequate margins. Wide excision with intraoperative frozen biopsy and Mohs micrographic surgery are common methods; however, these are associated with a high recurrence rate and long operation time, respectively. METHODS: Between January 2010 and June 2013, 21 patients diagnosed with EMPD underwent mapping biopsy. Biopsy specimens were collected from at least 10 areas, 2 cm from the tumor margin. When the specimens were positive for malignancy, additional mapping biopsy was performed around the biopsy site of the positive result, and continued until no cancer cells were found. Based on the results, excision margins and reconstruction plans were established preoperatively. RESULTS: The patients (18 male, 3 female) had a mean age of 66.5 years (range, 50-82 years). Almost all cases involved in the perineal area, except one case of axillary involvement. Permanent biopsy revealed one case (4.8%) of positive cancer cells on the resection margin, in which additional mapping biopsy and re-operation was performed. At the latest follow-up (mean, 27.4 months; range, 12-53 months), recurrence had not occurred. CONCLUSIONS: Preoperative mapping biopsy enables accurate resection margins and a preoperative reconstructing plan. Additionally, it reduces the operation time and risk of recurrence. Accordingly, it represents an effective alternative to Mohs micrographic surgery and wide excision with intraoperative frozen biopsy.

Case for diagnosis / Caso para diagnostico

An 83 year-old man was observed for an 8 year-long pruritic, erythematous plaque of the left axilla. He had been continuously medicated with topical antifungals and steroids with occasional symptomatic relief, but with disease progression. After a clinical and histological diagnosis of Extramammary Paget's Disease, a total excision with wide margins was performed. He remains disease-free 12 months after surgery. We report this rare presentation of Extramammary Paget's Disease on a male patient.

Um homem de 83 anos foi observado por placa eritematosa pruriginosa da axila esquerda com 8 anos de evolução. Estivera continuamente medicado com antifúngicos e corticóides tópicos com alívio sintomático ocasional, contudo com progressão da doença. Após diagnóstico clínico e histológico de Doença de Paget Extramamária, foi feita excisão total com margens largas. Mantém-se sem evidência de doença aos 12 meses após cirurgia. Salientamos esta apresentação rara de Doença de Paget Extramamária num paciente do sexo masculino.

Enfermedad de Paget extensa de la vulva: Presentación de un caso y propuesta de manejo conservador / Extensive Paget's disease of the vulva: Case report and a conservative management proposal

La enfermedad de Paget extramamaria (EPE) es una patología maligna observada, sobre todo, en áreas donde existe alta densidad de glándulas apocrinas. Presentamos el caso clínico de una paciente de 68 años de edad, atendida en la Clínica de Colposcopia del Servicio de Oncología, del Instituto Nacional de Perinatología Isidro Espinosa de los Reyes, con diagnóstico de enfermedad de Paget a nivel vulvar, vagina, región de periné y región perianal, manejada con sesiones de vaporización con láser, con evolución satisfactoria. Dado que es una enfermedad poco frecuente, más común en el sexo femenino y en la raza blanca, con mayor incidencia en la séptima década de la vida, como es el caso de nuestra paciente, el objetivo de este trabajo es presentar una revisión de la evaluación clínica, haciendo énfasis en el diagnóstico diferencial, uso de colposcopia para diagnóstico y tratamiento conservador fraccionado con vaporización de láser. La baja incidencia de la enfermedad de Paget de la vulva, vagina, periné y región perianal, así como la ausencia de informes sobre la posibilidad de tratamiento no quirúrgico, motivan a informar a la comunidad científica sobre el manejo con vaporización láser, con la intención de evitar la morbilidad que puede traer consigo el manejo quirúrgico convencional.

Extramammary Paget's disease (EPD) is a malignant disease observed, especially in areas with high density of apocrine glands. We report a case of a patient of 68 years old, attended at the Colposcopy Clinic of Oncology, National Institute of Perinatology Isidro Espinosa de los Reyes, diagnosed with Paget's disease of the vulva, vagina, perineum region and perianal region, and managed with laser vaporization sessions with satisfactory outcome. Since it is a rare disease, more common in females and in whites, with the highest incidence in the seventh decade of life, as is the case of our patient, propose in this paper to present a review of the clinical evaluation with emphasis on the differential diagnosis, use of colposcopy for diagnosis and dividing up conservative treatment with laser vaporization of the disease. The low incidence of Paget's disease of the vulva, vagina, perineum and perianal region, and the absence of reports on the possibility of nonsurgical treatment encouraged to inform the scientific community about the management with laser vaporization, with the intention of avoiding morbidity that can lead to the conventional surgical maneuvers.