Desmoplastic Trichoepithelioma: A Case Report with Familial Context

Abstract

Background: Desmoplastic trichoepithelioma (DTE) is a rare, benign adnexal tumor derived from follicular germinative cells. It predominantly affects young females and commonly presents on the face as annular plaques or papules with a depressed center. The tumor poses diagnostic challenges due to its resemblance to malignant lesions such as morpheaform basal cell carcinoma (BCC) and microcystic adnexal carcinoma (MAC). Diagnosis is confirmed by histological examination. Case Report: We report the case of a 29-year-old woman presenting with multiple facial DTEs and a similar presentation in her sister, suggesting a familial predisposition. Discussion: Desmoplastic trichoepithelioma can closely mimic aggressive skin cancers both clinically and histologically, necessitating biopsy for accurate diagnosis. The unusual occurrence of multiple lesions and familial involvement in this case raises the possibility of a genetic predisposition, although this remains rare. Early recognition is essential to avoid overtreatment. Conclusion: This case highlights the importance of considering DTE in the differential diagnosis of facial papules, especially when lesions are multiple or familial, and confirms the role of histology in establishing a definitive diagnosis.