Explanation

Paget disease of the vulva is a rare form of intraepithelial neoplasia. The most common presentation is pruritus, and it is often confused with other more common skin conditions, leading to a delay in diagnosis. Physical exam findings will show either an erythematous lesion, where the vulva epithelium is red or violet, or an eczematoid lesion, where the red skin is covered with a white coating sometimes described as cupcake frosting in appearance. Most patients with Paget disease of the vulva will have a preinvasive state, but an underlying adenocarcinoma may be present in rare cases. Diagnosis is made with biopsy, and treatment is wide local excision.

Classically, lichen planus (A) presents with lesions that are white, reticular, lacy, or fern-like, also known as Wickham striae. Pink or purple papules without clear borders may also be present. Itching, along with dyspareunia, burning, or soreness, are common symptoms. Lichen sclerosus (B) presents as white papules or plaques with areas of ecchymosis or purpura, rather than a wider area of white coating seen with Paget disease. The skin is often thin, white, or crinkled like cigarette paper. Common symptoms are pruritus, dyspareunia, burning, and tearing. Lichen simplex chronicus (C) presents as one or more erythematous scaling plaques with irregular borders. In long-standing disease, the skin appears thickened and leathery, or bark-like in appearance. Excoriations are usually visible due to the severe pruritus associated with the condition.