Introduction/Background Occurring as a rare cutaneous malignancy, Extramammary Paget’s Disease manifests as a predilection on the genital and perianal areas and is associated with underlying carcinoma of adjacent organs.

The pathophysiological mechanism is still unclear, and the clinical presentation of the lesions often demonstrate infiltrative erythema similar with that of other skin disorders like eczema. Primarily affecting the elderly, post-menopausal women, and Asians.

The disease is notorious due to its chronic relapsing clinical course. There are no established guidelines that exist in terms of management for EMPD. Surgery remains the treatment of choice but the procedure is usually extensive and carries a high recurrence rate.

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Abstract #157 Figure 1

Methodology This is a case of a 43-year-old female patient, diagnosed as a case of Extramammary Paget’s Disease of the Vulva in 2014. The patient underwent radical wide excision and split thickness skin graft 5 years after the symptoms started. Final histopathological examination with panel of immunohistochemical stains confirmed the diagnosis of Primary Cutaneous Paget’s Disease of the Vulva.

Results Patient was clinically stable for 4 years, but patient noted recurrence of previous symptoms such as rashes/redness on the vulvar area and pruritus with associated joint pains, body weakness and loss of appetite.

On presentation, the entire vulva was converted into a hyperkeratotic eczematous plaque with ‘weeping’ appearance at the vulva and perineum. Vulvar Biopsy with immunostaining confirmed findings compatible with Paget’s Disease.

Bone Scintigraphy revealed multiple areas of metastasis. Whole Abdominal Ultrasound showed slight hepatomegaly probably metastasis.

Conclusion Though the risk of developing of metastasis even after treatment is very low, the risk of recurrence after standard surgical treatment is still high. Proper diagnosis is very crucial, and definitive treatment should have been offered.

A wider knowledge and better understanding of the presentation, diagnosis and management of the disease should be developed in future studies

Disclosures The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.