Harlequin Ichthyosis (IH), Autosomal Recessive Manifestation, A Review Article

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منابع مشابه

Autosomal recessive congenital ichthyosis.

The term autosomal recessive congenital ichthyosis (ARCI) refers to a group of rare disorders of keratinization classified as nonsyndromic forms of ichthyosis. This group was traditionally divided into lamellar ichthyosis (LI) and congenital ichthyosiform erythroderma (CIE) but today it also includes harlequin ichthyosis, self-healing collodion baby, acral self-healing collodion baby, and bathi...

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Harlequin ichthyosis and other autosomal recessive congenital ichthyoses: the underlying genetic defects and pathomechanisms.

Autosomal recessive congenital ichthyoses (ARCI) include several severe subtypes including harlequin ichthyosis (HI), lamellar ichthyosis and non-bullous congenital ichthyosiform erythroderma. Patients with these severe types of ichthyoses frequently show severe hyperkeratosis and scales over a large part of the body surface form birth and their quality of life is often severely affected. Recen...

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Molecular Genetics of Autosomal Recessive Congenital Ichthyosis

3 It seems as though I had not drunk from the cup of wisdom, but had fallen into it Søren Kierkegaard To Panu

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Oral Manifestation of Autosomal Recessive Congenital Ichthyosis in a 2-Year-Old Patient

Ichthyosis is a heterogeneous family of hereditary keratinisation disorders mostly characterized by variable erythema of the whole body and different scaling patterns. Although these disorders affect tissues of epidermal origin, there is little evidence regarding the oral and dental manifestations of Lamellar Ichthyosis. A case report of early childhood caries in lamellar ichthyosis is presente...

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Harlequin ichthyosis (or harlequin fetus)

The pat ient was a female neonate bor n prematurely on 36 weeks of gestation by normal vaginal delivery. During pregnancy, mother had no history of fever, drug use or x-ray exposure. The mother was gravida 2 and had no history of abortion. Her first baby was well. On physical examination, the skin was markedly thickened, hard and hyperkeratotic with deep crevices running transversely and vertic...

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ژورنال

عنوان ژورنال: Biomedical Journal of Scientific & Technical Research

سال: 2021

ISSN: 2574-1241

DOI: 10.26717/bjstr.2021.34.005547