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

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...

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Published in:Medicina (Kaunas, Lithuania) Lithuania), 2021-10, Vol.57 (10), p.1029
Main Authors: Kosmidis, Christoforos S., Sevva, Christina, Roulia, Panagiota, Koulouris, Charilaos, Varsamis, Nikolaos, Koimtzis, Georgios, Theodorou, Vasiliki, Mystakidou, Chrysi Maria, Georgakoudi, Eleni, Anthimidis, Georgios
Format: Article
Language:English
Subjects:
Biopsy
Case Report
Case reports
Cytokeratin
Dermatitis
extramammary
General anesthesia
Gynecology
Hemodialysis
Histopathology
Magnetic resonance imaging
Melanoma
Paget’s disease
Patients
Pruritus
Skin cancer
Tumors
vulva
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Summary: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.
ISSN:1648-9144
1010-660X
1648-9144
DOI:10.3390/medicina57101029