[Familial dyskeratotic comedones. A rare entity].

Familial dyskeratotic comedones is a rare autosomal dominant genodermatosis. In childhood or adolescence disseminated keratotic papules develop and gradually increase in number with time. The isolated papules show a central keratotic plug which tends to recur after extraction. Pruritus and occasional inflammation are the only symptoms. The lesions appear on the extremities, and less frequently on the trunk and the face. About half of the patients have a history of acne vulgaris. The histologic picture is highly characteristic and shows a deep invagination of an acantholytic and dyskeratotic epidermis with prominent cornification. Familial dyskeratotic comedones are generally refractory to any therapy. We report on two sisters with familial dyskeratotic comedones successfully treated by CO2-laser therapy.