This is an autosomal dominant condition. It is characterized by multiple, smooth, dome-shaped, firm, pink Tumours of the head (turban Tumours), including syringomas and trichoepitheliomas, and these may be very disfiguring. Basal cell epitheliomas may develop in the lesions, and radiation therapy may exacerbate this tendency.
The Tumours are of hairy skin and are not associated with basal or squamous cell carcinomas. The condition has been linked to D16S411 and D16S416 on chromosome 16q12–13.