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Two sisters with familial dyskeratotic comedones


European Journal of Dermatology. Volume 14, Number 4, 214-5, July-August 2004, Genes and Skin

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Author(s) : Christian HALLERMANN, Hans Peter BERTSCH

Summary : 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.

Keywords : comedones, familial dyskeratotic comedones

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