Trichilemmal Carcinoma in a Patient With Oculocutaneous Albinism.

Trichilemmal carcinoma (TC) is a rare adnexal neoplasm derived from the outer sheath of the hair follicle. Despite its aggressive histological features, TC typically exhibits low-grade clinical behavior, with local recurrences being more common than metastasis. This case report describes a 47-year-old Hispanic female with oculocutaneous albinism and a history of numerous nonmelanoma skin cancers (NMSCs) who presented with an erythematous papule on her forearm. Histopathology revealed TC characterized by basaloid keratinocytes with clear cytoplasm and peripheral palisading and a high proliferation rate confirmed by Ki-67. Surgical excision with 1.0 cm margins was performed, and final histopathology showed no residual tumor. Concurrently, the patient was diagnosed with metastatic squamous cell carcinoma (SCC) and started on pembrolizumab. This case highlights the increased susceptibility of individuals with oculocutaneous albinism to multiple and varied skin malignancies, stressing the need for vigilant skin surveillance and a broad differential diagnosis in this population. The patient's history underscores the importance of early detection and intervention to manage and mitigate the risk of advanced skin cancers.