Bullous Ichthyosiform Erythrodermia

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Bullous ichthyosiform erythroderma

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 corrugated...

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Non-bullous congenital ichthyosiform erythroderma.

A 4-day-old neonate was brought with complaints of fissuring and peeling of skin involving almost the whole body. The neonate had generalized desquamation not even sparing the palms, soles and face. The parents informed that the baby was born, enclosed in a constricting parchmentlike membrane (collodion baby) that had gradually comeoff. There was a past history of a child born with similar pres...

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Bullous congenital ichthyosiform erythroderma: safe and effective topical treatment with calcipotriol ointment in a child.

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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Dental Treatment of a Child Suffering from Non-bullous Congenital Ichthyosiform Erythroderma under General Anesthesia

Non-bullous congenital ichthyosiform erythroderma (NBCIE) is an autosomal recessive form of inherited icthyosis appears as fine white scales that gradually replace collodion membrane. This case report describes management of 5 years and 11-month-old child with NBCIE suffering from early childhood caries (ECC) under general anesthesia. How to cite this article: Choudhary R, Satish V. Dental Trea...

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ژورنال

عنوان ژورنال: Proceedings of the Royal Society of Medicine

سال: 1966

ISSN: 0035-9157

DOI: 10.1177/003591576605901017