Bullous ichthyosiform erythroderma
نویسندگان
چکیده مقاله:
Bullous ichthyosiform erythroderma (BIE) is a rare disorder of keratinization (mutations in either keratin 1 or 10). It typically presents with fragile skin, which gives way to gradual evolutionof hyperkeratosis. Flaccid blisters, peeling, and superficial erosions at sites of minor trauma or friction are apparent within the first few hours of life. Yellow-brown, waxy, ridged or corrugatedscales build up in skin creases, sometimes forming spiny (Hystrix) outgrowths. Cobble stone-like keratoses occur at other sites such as the dorsal aspects of the hands and feet and over the trunk.We report an 11-year-old boy with a generalized hyperkeratosis on the neck, trunk, extremities, and scalp.
منابع مشابه
Non-bullous congenital ichthyosiform erythroderma.
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Sir, Bullous congenital ichthyosiform erythroderma (BCIE), or epidermolytic hyperkeratosis (MIM# 113.800), is a rare disorder of keratinization associated with blistering in its early phase. It was first described by Jean-Louis Brocq in 1902 as érythrodermie congénitale ichthyosiforme avec hyperépidermotrophie. Although most cases are sporadic, familial cases show an autosomal dominant pattern ...
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عنوان ژورنال
دوره 16 شماره 3
صفحات 112- 115
تاریخ انتشار 2013-07-01
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