[Keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK syndrome)]. / Queratosis linear con ictiosis congénita y queratodermia esclerosante (síndrome KLICK).

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.

Queratosis linear con ictiosis congénita y queratodermia esclerosante (síndrome KLICK) / Keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK syndrome)

Describimos el caso de un paciente de 32 años de edad que desde el nacimiento presentaba dermatosis ictiosiforme generalizada, queratodermia palmoplantar con bandas constrictivas alrededor de los dedos y placas queratósicas de distribución lineal localizadas en grandes pliegues. El examen dermatopatológico evidenció hiperqueratosis ortoqueratósica e hiperplasia epidérmica con hipergranulosis. No existían otros miembros afectados en su familia. Con estos hallazgos clínicos e histológicos se diagnosticó como queratosis lineal con ictiosis congénita y queratodermia esclerosante (síndrome KLICK). El paciente rechazó el tratamiento con retinoides orales y fue tratado con emolientes y cremas de urea al 30 %, con escasa respuesta clínica

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