Multiple familial trichoepithelioma

A 25-year-old male presented with 12 years history of multiple firm, non-tender, shiny, skin-colored papules, and nodules of varying sizes of 2–10 mm over face [Figure 1 a-c]. Similar history of skin-colored lesions over face was present in his mother and maternal uncle. Rest of cutaneous and systemic examinations was normal. Based on the clinical and histopathological findings [Figure 1d], a diagnosis of trichoepithelioma was made.

Trichoepithelioma is a rare benign adnexal tumor which presents as solitary non-familial or multiple familial forms, predominantly over the face. Although rare, it can be associated with other follicular or appendageal tumors. The differential diagnosis includes basal cell carcinoma, Brooke-Spiegler syndrome, and trichoblastoma.^[1]

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Figure 1:

(a-c) Multiple trichoepitheliomas over face. (d) Histopathological examination showing multiple adnexal tumors (yellow arrow) comprised nests of basaloid cells and keratin cysts (black arrow) in the superficial dermis (H&E stain; ×20).

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