Epidermolytic hyperkeratosis: generalized form in children from parents with systematized linear form
V Nazzaro, E Ermacora, B Santucci… - British Journal of …, 1990 - academic.oup.com
V Nazzaro, E Ermacora, B Santucci, R Caputo
British Journal of Dermatology, 1990•academic.oup.comTwo unrelated families are presented in both of which a child with generalized epidermolytic
hyperkeratosis (congenital bullous ichthyosiform erythroderma) had a parent with linear
epidermolytic hyperkeratosis (epidermolytic epidermal naevus). Light and electron
microscopy of skin biopsies of lesions from the children and parents showed typical
epidermolytic hyperkeratosis. Gonadal mosaicism in patients with linear epidermolytic
hyperkeratosis may be responsible for transmission of the abnormality to the offspring.
hyperkeratosis (congenital bullous ichthyosiform erythroderma) had a parent with linear
epidermolytic hyperkeratosis (epidermolytic epidermal naevus). Light and electron
microscopy of skin biopsies of lesions from the children and parents showed typical
epidermolytic hyperkeratosis. Gonadal mosaicism in patients with linear epidermolytic
hyperkeratosis may be responsible for transmission of the abnormality to the offspring.
Summary
Two unrelated families are presented in both of which a child with generalized epidermolytic hyperkeratosis (congenital bullous ichthyosiform erythroderma) had a parent with linear epidermolytic hyperkeratosis (epidermolytic epidermal naevus). Light and electron microscopy of skin biopsies of lesions from the children and parents showed typical epidermolytic hyperkeratosis. Gonadal mosaicism in patients with linear epidermolytic hyperkeratosis may be responsible for transmission of the abnormality to the offspring.
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