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Case Reports

Abstract

Angiokeratomas are benign vascular lesions characterized by dilated dermal vessels with overlying epidermal hyperkeratosis. While reactive epidermal changes are common, true epithelial dysplasia is rare, and the occurrence of high‑grade squamous intraepithelial lesion (HSIL) within an angiokeratoma has not been widely documented. We report a case of a 30‑year‑old woman with Crohn’s disease and carrier status for X‑linked chronic granulomatous disease who developed progressive enlarging friable vulvar lesions. The initial biopsy demonstrated angiokeratoma, while subsequent excision revealed multifocal high-grade dysplasia within the epidermis overlying the angiokeratoma. The atypical squamous cells exhibited diffuse p63 and p16 expression, with high-risk HPV detected by in situ hybridization. These findings confirm the diagnosis of HPV-associated HSIL in conjunction with angiokeratoma. To the best of our knowledge, this is the first reported case of HPV-associated HSIL arising in conjunction with a vulvar angiokeratoma. Although these lesions are almost always benign and epithelial atypia is typically reactive, in this patient, underlying immune dysregulation may have impaired viral clearance, creating an environment prone to neoplastic transformation within a pre-existing vascular lesion. This case highlights the importance of thorough histologic evaluation of even clinically benign-appearing vascular lesions, particularly in individuals with chronic inflammatory or immune-mediated conditions, as such lesions may harbor significant pathological changes that warrant targeted management and surveillance.