A syndrome of trichoepitheliomas, milia, and cylindromas

Abstract

Three consecutive generations of a family have had members affected with trichoepitheliomas and milia. One member, the proband, also had cylindromas. The pattern of inheritance was a dominant one, with only the male members affected. Histopathologically, the trichoepitheliomas and milia contained keratinizing cysts with laminated centers, peripheral basaloid cells, and a thin granular layer. Fibroblastic stroma and fronding of basaloid cells were also seen in both tumors. The trichoepitheliomas and milia differed mainly in the greater proportion of keratinized cells found in the milia. Clinically, the milia were found only in areas of vellus hairs.