[Ichthyosis hystrix with parakeratosis in the form of cornoid lamellae]

We report on a keratinization disorder in four brothers in a family in which members had been affected in three generations. Clinical signs and genetic, histopathologic, autoradiographic and ultrastructural examinations all support the diagnosis of hystrixlike, proliferative ichthyosis with cornoid lamellae and autosomal dominant inheritance. The relationship of the disorder to other hystrixlike genetic keratinization disorders is discussed.