Multiple Familial Trichoepithelioma: Analysis Of Clinical Features

Multiple familial trichoepithelioma(MFT) is a benign skin tumor related to the pilosebaceous apparatus and inherited in autosomal dominant manner. Attentions have been focused on the mapping and mutation of the disease gene. But the clinical features of MFT are not clearly analyzed in Chinese.Through investigating a pedigree of MFT and reviewing sixteen families reports in the recent last sixteen years, we analyzed genetic type, clinical situation, histopathology and distinguished diagnose from familial cylindromatosis (FC) and basal cell carcinoma (BCC).Firstly, we diagnosed a patient with MFT as the proband. Secondly, we surveyed situation of pedigree of the proband and did clinic and genetics analysis after doing clinic and genetics analysis. Thirdly, we reviewed 16 reports about MFT pedigrees published in the recent 16 years.Clinical features of MFT were conclued: (DMFT is at autosomal dominant pattern. (2)The involvement of MFT in woman is more than that in man. (3)The patients showed multiple dome-shaped, skin-colored, firm papules on the face. Some patients also present the lesions on the scalp and neck. No lesion showed the malignant tendency. (4) The histologic findings of MFT are cornified cysts and some nests of basaloid epithelial cells. (5)There were variable clinical expressions in different patients of the same family. (6) Some pedigrees were also affected by feckle or symmetrical progressive erythrokeratoderma, which were not found in MFT patients of other origin.Multiple familial trichoepithelioma is inherited in autosomal dominant manner with high penetrance. The involvement of MFT in woman is more than that in man. Multiple lesions commonly present small tumors located predominantly in the nalolabial folds symmetrically. Most of the tumors are dome-shaped, skin-colored, firm...