Anjum Kanjee, Sabina Saeed, Javed Wasim, Ghous Qurieshi.
Trichoepitheliomas and Cylindromas in three generations of a family.
J Pak Assoc Derma Jan ;12(1):51-3.

Trichoepitheliomas is an uncommon benign tumor of immature hair structures, which is deeply situated in dermis. It is an autosomal dominant condition characterized by multiple tumors, which usually appear at puberty. Individual tumors reach a limiting size, but continue to increase in numbers. The simultaneous occurrence of trichoepitheliomas and cylindromas has been observed on numerous occasions. We report a family who presented with multiple tumors, which were confirmed to be trichoepitheliomas and cylindromas on histopathological examination. These tumors caused a gross disfiguration. The larger tumors were excised.

Case Reports: Marzia a 48-year-old female presented with multiple papular and nodular lesions involving her face, especially the nose, ears, forehead and upper eyelid. The lesions were also present on her scalp, chest, trunk and upper arm. These lesions appeared at the time of puberty and have been increasing in number and size since then. The lesions were skin colored and of varying sizes. There was a pinkish nodule on the forehead showing telangiectatic vessels on the surface. A bluish multinodular lesion was present on the front of neck and a larger nodule measuring about 9cms x 8cms was present on the back of neck. The family history revealed that the patient had three brothers but only one was affected with extensive disease. Her mother was also affected, however her father did not suffer from the disease. She is unmarried. Her affected brother has 5 children, 4 daughters and one son. His 3 daughters suffer from the same disease. Skin biopsies revealed multiple trichoepitheliornas and cylindromas. The larger tumors causing cosmetics disfigurement were excised.

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