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Case Reports
. 2021 Sep 27;57(10):1029.
doi: 10.3390/medicina57101029.

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

Christoforos S Kosmidis  1   2 , Christina Sevva  3 , Panagiota Roulia  2 , Charilaos Koulouris  1   2 , Nikolaos Varsamis  1 , Georgios Koimtzis  4 , Vasiliki Theodorou  3 , Chrysi Maria Mystakidou  3 , Eleni Georgakoudi  1 , Georgios Anthimidis  1
Affiliations
Case Reports

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

Christoforos S Kosmidis et al. Medicina (Kaunas). .

Abstract

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.

Keywords: Paget’s disease; case reports; extramammary; vulva.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Preoperative images of the lesion showing the erythematous plaque with white patches (a) and the extent of the WLE (b).
Figure 2
Figure 2
Images of the umbilical nodule upon clinical examination (a), the surgical excision of the nodule (b), and the final specimen (c).
Figure 3
Figure 3
Intraoperative images of the first specimen of the WLE (a,b) and the final plastic reconstruction of the skin (c).
Figure 4
Figure 4
Histopathological and Immunohistochemistry images: Intraepidermal atypical epithelioid cells H&E × 100 (a); intraepidermal atypical epithelioid cells with moderate to severe nuclear atypia H&E × 200 (b); the tumor cells are positive for CK7 immunostaining × 200 (c); the tumor cells are negative for cocktail HMB45/melanA. Positivity is observed only in normal melanocytes immunostained × 200 (d).
Figure 5
Figure 5
Preoperative images showing the typical white plaque and the erosions (a) and the extent of the WLE (b).
Figure 6
Figure 6
Intraoperative images of the second patient’s WLE (a) and the final plastic reconstruction of the skin (b).
Figure 7
Figure 7
Intraepidermal atypical epithelioid cells H&H × 100 (a); intraepidermal atypical epithelioid cells with moderate to severe nuclear atypia H&H × 200; (b) the tumor cells, positive for CK AE1/AE3 immunostaining × 200 (c); the tumor cells, negative for cocktail HMB45/melanA. Positivity is observed only in normal melanocytes immunostained × 200 (d).
See this image and copyright information in PMC

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