Epidermolytic hyperkeratosis (bullous
ichthyosiform erythroderma)
This
rare condition is inherited as an autosomal dominant disorder. Shortly after
birth the baby’s skin becomes generally red and shows numerous blisters. The
redness fades over a few months, and the tend-ency to blister also lessens, but
during childhood a gross brownish warty hyperkeratosis appears, sometimes in a
roughly linear form and usually worst in the flexures. The histology is
distinctive: a thickened granular cell layer contains large granules, and
clefts may be seen in the upper epidermis. The condition is caused by mutations
in the genes (on chromosomes 12q13 and 17q21) controlling the production of
keratins 1 and 10. A few patients with localized areas of hyperkeratosis with
the same his-tological features have gonadal mosaicism, and so their children
are at risk of developing the general-ized form of the disorder. Treatment is
symptomatic and antibiotics may be needed if the blisters become infected.
Acitretin has helped in severe cases.
Related Topics
Privacy Policy, Terms and Conditions, DMCA Policy and Compliant
Copyright © 2018-2024 BrainKart.com; All Rights Reserved. Developed by Therithal info, Chennai.