[Development of cancer (vulvar cancer) in the Netherton syndrome (ichthyosis, hair anomalies, atopic diathesis)].

So far there has been only one incidence of a malignant tumour associated with Netherton's syndrome, a rare skin disease consisting of congenital ichthyosiform erythroderma, atopic dermatitis and trichorrhexis nodosa (bamboo hair). The aetiology and clinical consequences are discussed on the occasion of a carcinoma of the vulva occurring in a case of a patient with Netherton's syndrome, both phenomena being interlinked.