Two sisters with familial dyskeratotic comedones

Abstract

Familial dyskeratotic comedones is a rare autosomal dominant genodermatosis, which presents with disseminated keratotic papules and comedo-like lesions. The disease usually manifests in childhood. The histology of the lesions is characterized by a crater-like invagination of the epidermis filled with parakeratotic keratin. The epidermis usually shows acantholysis. So far the causative gene defect is unknown. We report on a new family with two affected sisters. The more severely affected woman was treated with oral retinoids without any effect. Afterwards some regions were successfully treated by CO(2) laser-therapy.