[Keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK syndrome)].

We describe the case of a 32-year-old male patient who had presented from birth with generalized ichthyosiform dermatosis, palmoplantar keratoderma with constrictive bands around the fingers and keratotic plaques in a linear arrangement, located in the large skin folds. The dermatopathological examination showed orthokeratotic hyperkeratosis and epidermal hyperplasia with hypergranulosis. No other members of the patient's family were affected. With these clinical and histological findings, the diagnosis was keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK syndrome). The patient rejected treatment with oral retinoids and was treated with emollients and 30 % urea creams, with little clinical response.