A family with palmoplantar epidermolytic hyperkeratosis

Familial epidermolytic hyperkeratosis confined to the palms and soles was first characterized by Klaus and Weinstein in 1970. This entity has been the subject of only four subsequent reports. We report a family previously diagnosed as suffering from tylosis (Thost Unna syndrome), in which eleven members have been affected, and review the literature on this disease. It would seem possible that this entity may be more common than the sparse literature would indicate, and that other cases may currently be masquerading as tylosis.