Multiple Familial Trichoepithelioma with Malignant Transformation
A 38-year-old female presented with a 27 years history of asymptomatic, skin colored papules. Lesions were firm in consistency, ranging from 5 to 10 mms in diameter and were distributed on her face; around eyes, on the nose, nasolabial folds and upper lip [Figure and ]. General examination revealed no abnormality. Dermatological examination revealed normal skin and mucous membranes. Patient's father had similar lesions but there was no affection of other family members. Routine investigations including hemogram, urine analysis, liver and renal function tests were non contributary.\nExcisional biopsy of a representative lesion was done after taking patient consent. Histopathological examination of hematoxylin and eosin (H and E)-stained sections revealed superficial dermal, well circumscribed, non capsulated, symmetrical lesion. This lesion was formed of solid aggregates of uniform basaloid cells with peripheral palisading but lacked epidermal connection, pilar differentiation and retraction artifacts. They were surrounded by a stroma with increased number of fibroblasts []. Cells were uniform, with large nuclei and scanty cytoplasm and lacked cytological atypia, mitoses or necrosis []. Aggregations of fibroblasts, representing abortive attempts to form papillary mesenchyme (papillary mesenchymal bodies), were detected, that are characteristic of TE []. Small foreign body granuloma was detected [].\nBased on clinical and histopathological criteria, the diagnosis of immature TE was reached. However the presence of solid basaloid aggregates with peripheral palisading may lead to a misdiagnosis of BCC. Thus, we performed immunohistochemical (IHC) staining for Bcl2, CD10, CD34 and androgen receptor (AR) antibodies. The outermost epithelial cells showed positivity for Bcl2 []. Both CD10 and CD34 stained the stromal cells but not the basaloid cells [Figure and ]. Both tumor cells and stroma did not show any AR immunoreactivity []. The final diagnosis was TE. The patient was treated by CO2 laser with no recurrence for one year.