A Case of Recurrent Vulvar Paget’s Disease Masquerading as Lichen Simplex Chronicus

Vulvar Paget’s disease (VPD) is a type of extramammary Paget’s disease that generally presents as an erythematous plaque. We report an atypical presentation of recurrent vulvar Paget’s disease presenting as a hyperpigmented plaque with a leucokeratosis surface on the vulva incompletely encircling the vaginal orifice and involving both inner lip and outer lip of the vagina mimicking lichen simplex chronicus. The biopsy was positive for Pagetoid cells, and immunohistochemistry was positive for cytokeratin (CK)7 and GATA binding protein 3 and negative for CK20, confirming the diagnosis of VPD (primary cutaneous type). We report this case for its rarity and atypical presentation, making the diagnosis delayed. Hence, a thorough idea of the approach to the case will help the dermatologist in early suspicion and diagnosis of a case like this for better management.