Familial dyskeratotic comedones. A rare entity

Familial dyskeratotic comedones is a rare autosomal dominant genodermatosis . In childhood or adolescence disseminated keratotic papules develop and gr adually increase in number with time. The isolated papules show a central k eratotic plug which tends to recur after extraction. Pruritus and occasiona l 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 characte ristic and shows a deep invagination of an acantholytic and dyskeratotic ep idermis with prominent cornification. Familial dyskeratotic comedones are g enerally refractory to any therapy. We report on two sisters with familial dyskeratotic comedones successfully treated by CO2-laser therapy.