Abstract

Trichoepithelioma is a benign skin tumor with folliculosebaceous differentiation. Although it usually occurs as solitary adnexal tumor, multiple familial and non-familial forms can also exist. Multiple Familial Trichoepithelioma (MFT) may develop as one of subtypes of Brooke-Spiegler Syndrome (BSS). Here, we describe a patient with multiple trichoepitheliomas. Patient is a 56-years old African-American female presented with multiple, skin colored, firm papulonodular lesions on her face, especially involving the nasolabial folds, upper lip, eyebrows and mid forehead. She states that the lesions continue to appear for the last 17 years. She also adds that she has visited multiple dermatologists and was diagnosed with BSS a few years prior to her current presentation. However, careful questioning of family history is unremarkable. Excisional biopsy from the upper lip is first reported as “Basal Cell Carcinoma”. After reviewing the clinical history on medical records, pathologic diagnosis is revised as “Trichoepitheloma”. On microscopy, Hematoxylin and Eosin stained slides reveal numerous horn cysts within the dermis and lobules of basaloid cells. Admixed with the horn cysts are lobules of basophilic tumor cells with minimal cytoplasm showing peripheral palisading, indistinguishable from those features of basal cell carcinoma. This morphology is most consistent with trichoepithelioma combined with the questionable history of BSS on this patient. Multiple trichoepitheliomas can occur as familial or non-familial forms. Without family history, it is more appropriate to categorize these multiple tumors as non-familial. Daily dermatopathology practice can be hectic with no time to dive deep in patients’ clinical history on medical records. However, this case to remind us to take time to question the diagnosis and better communicate with the clinician.

Financial Disclosure:
No current or relevant financial relationships exist.