Vulvar Verruciform Xanthoma

Verruciform xanthoma (VX) is a rare benign tumor that usually involves the oral cavity. Since the first report of this tumor in 1971, only 9 cases have been reported on the vulva, and 3 of these were associated with another vulvar condition. We describe the clinicopathologic features of 10 patients with vulvar VX and focus on their associated conditions.The mean age of the patients was 68 years (range, 51-80 years). The VX lesions were asymptomatic, yellowish-orange verrucous plaques. The diagnosis was clinically suspected in 2 cases; other suggested diagnoses were condyloma or squamous cell carcinoma. All of the patients had an associated vulvar condition: lichen sclerosus (6 patients), lichen planus (2 patients), Paget disease, or radiodermatitis. Under microscopy, the VX lesions displayed parakeratosis, acanthosis without atypia, and elongated rete ridges. Xanthomatous cells were aggregated in the papillary dermis.Vulvar VX is a benign tumor with misleading clinical features. All 10 cases were associated with a vulvar condition, mainly a lichen sclerosus. Therefore, VX might represent a reaction pattern induced by different conditions, mainly characterized by damage to the dermoepidermal junction. When confronted with the diagnosis of vulvar VX, clinicians may look for an associated vulvar condition.