scholarly journals Extramammary Paget’s Disease of the Vulva: Report of Two Cases

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1029
Author(s):  
Christoforos S. Kosmidis ◽  
Christina Sevva ◽  
Panagiota Roulia ◽  
Charilaos Koulouris ◽  
Nikolaos Varsamis ◽  
...  

Extramammary Paget’s disease is a rare condition, affecting 6.5% of all patients with Paget’s disease. The most common extramammary site is the vulvar area. Although diagnosis in some patients is difficult to set, early diagnosis is of high importance in order to detect the irreversible progression of the lesion early and prevent distant metastasis. An 89-year-old female and a 69-year-old female presented within three months with an eczematous lesion with leukoplakia in the vulva. The incisional biopsy of the skin revealed extramammary Paget’s disease. Both patients underwent a surgical wide local excision of the lesion and the specimens were sent for histopathological examination. Extramammary Paget’s disease has a high potential for distant malignancies and local recurrence, dictating that surgical excision is the most efficient treatment. The rareness of the condition and the diagnostic difficulties underline the need for early skin biopsy, which is the most efficient diagnostic tool.

2013 ◽  
Vol 5 (4) ◽  
pp. 171-176
Author(s):  
Slobodan Stojanović ◽  
Nada Vučković ◽  
Pavle Jeremić ◽  
Biljana Jeremić

Abstract The authors present a case of a patient with extramammary Paget’s disease in the pubic region treated by a dermatologist with a private practice for almost 4 years before incisional biopsy was performed. A thorough examination showed no evidence of malignancies of internal organs, whereas definite diagnosis was made by excision of the entire skin lesion and histopathological analysis. With regard to multiple criteria, findings in our patient were consistent with current literature data, but interestingly, the primary extramammary Paget’s disease has not spread into deeper tissues.


2016 ◽  
Vol 9 (1) ◽  
pp. 6-10 ◽  
Author(s):  
Yuki Yoshihara ◽  
Munenari Itoh ◽  
Yoshimasa Nobeyama ◽  
Hidemi Nakagawa

Extramammary Paget's disease (EMPD) is an uncommon cutaneous adenocarcinoma arising from the apocrine glands within the epidermis or underlying skin appendages in the anogenital and axillary regions. Surgical excision is basically performed as a treatment for EMPD. However, therapeutic options for EMPD in an advanced stage are limited. Herein, we report the case of a Japanese woman with advanced EMPD successfully controlled by monthly but not weekly docetaxel therapy. We also demonstrate the possibility that a monthly regimen of docetaxel is a more effective and optimal schedule than a weekly one through this case report.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Rosa Guerra ◽  
Subhasis Misra

Extramammary Paget's Disease (EMPD) is a rare condition of the skin that often involves the vulva, perianal region, scrotum, penis, and axilla. Although prognosis is generally favorable, it can be associated with neoplasms of the bladder, urethra, prostate, and rectum. This report presents a case of scrotal EMPD that failed treatment with imiquimod 5% cream and discusses benefits and complications of available treatment options. The variation of treatment success emphasizes the importance of further research.


2021 ◽  
Vol 5 (1) ◽  
pp. 68-70
Author(s):  
Roxana Mititelu ◽  
Claudia Flores-Echaiz ◽  
Manish Kanna

Introduction: We report an elderly gentleman with Extramammary Paget’s disease (EMPD) treated with Mohs micrographic surgery (MMS) using Toluidine blue staining intraoperatively as to detect the Paget’s cells. Case Presentation: An elderly man presented with an erythematous plaque on the left inguinal fold which showed in-situ EMPD on histopathological examination. Investigations for secondary EMPD were negative and the patient was treated with MMS. During MMS, the specimens from the patient were stained using Toluidine blue in order to detect the Paget cells and to determine the appropriate negative margin. At 4 years follow up the patient is free of recurrence. Conclusion: Toluidine blue is a fast, user-friendly dye that can be used intraoperatively during MMS as to detect Paget cells and thus to determine the appropriate negative margin.


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