Vulvar Paget's disease associated with squamous cell carcinoma: A case report

INTRODUCTION: and Importance: Extra Mammary Paget's disease (EMPD) of the vulva, a rare postmenopausal entity, is responsible for less than 1% of all vulvar neoplasms. Invasive EMPD of the vulva with underlying squamous cell carcinoma is even rare. CASE PRESENTATION: A 70-year-old para 5 postmenopausal lady presented with a history of vulvar itching and a gradually progressive reddish lesion on genitals unresolved by topical therapies for one year. Vulvar biopsy confirmed the presence of pagetoid cells with a focus of squamous invasion. DISCUSSION: The clinical presentation is often non-specific and typically presents as a pruritic skin rash in the vulva. Optimal management of EMPD of the vulva is unclear, but wide surgical excision is considered the standard therapeutic approach. Local recurrence in EMPD is common even with aggressive radical procedures. Constant follow-up is required to ensure early diagnosis of recurrences. CONCLUSION: Early biopsy of the suspicious eczematous lesion can help in definitive diagnosis and timely treatment of EMPD.