Bazex syndrome. Follicular atrophoderma and basal cell epitheliomas

A dominant inheritance leading to development of nevoid basal cell epitheliomas on the face and follicular atrophoderma on the extremities constitute the two constant manifestations of Bazex syndrome. A patient with both manifestations on the trunk who fulfilled all the criteria for a diagnosis of nevoid basal cell epitheliomas is described. Histopathology of the basal cell cancer corresponded to that of the fibroepithelial tumor, thus differing from the more common nodular and trichoepitheliomatous presentations. Difficulty in associating the atrophoderma with a hair follicle (histologically) arises because the latter ceases to exist as a functional unit.