Congenital ichthyosiform erythroderma is an autosomal recessive genetic disorder. This means a child must inherit a defective pair of genes (one from each parent) to show the symptoms. Parents who are carriers of the defective genes show no symptoms but their children have a 25% chance of having the disease.

Nonbullous congenital ichthyosiform erythroderma (NBCIE) is a condition that mainly affects the skin. Many infants with this condition are born with a tight, clear sheath covering their skin called a collodion membrane.

Epidermolytic ichthyosis is one of the five main types of ichthyosis along with lamellar ichthyosis, ichthyosis vulgaris, congenital ichthyosiform erythroderma, and X-linked ichthyosis. It typically presents at birth with erythroderma, skin fragility, and blistering [1–3].

Nonbullous congenital ichthyosiform erythroderma (NBCIE) is a specific type of ichthyosis mainly affecting the skin. Most infants with NBCIE are born with a tight, shiny covering on their skin, called a collodion membrane, which is typically shed within a few weeks.

Congenital ichthyosiform erythroderma (CIE) is a generalized skin disease in which a more or less pronounced erythroderma predominates. A collodion membrane may sometimes be present at birth and will change into ichthyosiform erythroderma after a few days.

2019年3月13日 · Congenital ichthyosiform erythroderma (CIE) may present in the newborn period as a collodion baby (milder than with lamellar ichthyosis) with ectropion and eclabium (Figure 1). As the collodion membrane is shed, patients develop diffuse erythema and fine, powdery scaling and sometimes thickening of the palms and soles.

A variant of autosomal recessive congenital ichthyosis (ARCI), a rare epidermal disease, characterized by fine, whitish scales on a background of erythematous skin over the whole body.

Ichthyosis with confetti (IWC), also known as congenital reticular ichthyosiform erythroderma (CRIE), is a rare skin condition characterized by slowly enlarging islands of normal skin surrounded by erythematous ichthyotic patches in a reticulated pattern.

CIE is sometimes referred to by the term non-bullous congenital ichthyosiform erythroderma (n-CIE) to distinguish it from bullous congenital ichthyosiform erythroderma, which shows additional features of skin fragility and is due to dominantly inherited mutations in keratin proteins.

CIE is part of the group of ichthyoses known as Autosomal Recessive Congenital Ichthyoses, known by the acronym ARCI. This is a varied group and includes other ichthyoses such as Lamellar ichthyosis (LI) and the more severe Harlequin Ichthyosis (HI).