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Erythroderma is an inflammatory skin disease with redness and scaling that affects nearly the entire cutaneous surface. [ 1 ] [ 2 ] This term applies when 90% or more of the skin is affected. In ICD-10 , a distinction is made between "exfoliative dermatitis" at L26, and "erythroderma" at L53.9.
Psoriatic erythroderma can be congenital or secondary to an environmental trigger. [12] [13] [14] Environmental triggers that have been documented include sunburn, skin trauma, psychological stress, systemic illness, alcoholism, drug exposure, chemical exposure (e.g., topical tar, computed tomography contrast material), and the sudden cessation of medication.
Epidermolytic hyperkeratosis (also known as "Bullous congenital ichthyosiform erythroderma," [7] "Bullous ichthyosiform erythroderma," [8]: 482 or "bullous congenital ichthyosiform erythroderma of Brocq" [9]) is a rare skin disease in the ichthyosis family, affecting around 1 in 250,000 people.
Less severe mutations result in a collodion membrane and congenital ichthyosiform erythroderma-like presentation. [ 15 ] [ 16 ] ABCA12 is an ATP-binding cassette transporter (ABC transporter), which is a member of a large family of proteins that hydrolyze ATP to transport cargo across cell membranes.
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.
Ritter's disease of the newborn is the most severe form of SSSS, with similar signs and symptoms. SSSS often includes a widespread painful erythroderma, often involving the face, diaper, and other intertriginous areas. Extensive areas of desquamation might be present. Perioral crusting and fissuring are seen early in the course.
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Keratitis–ichthyosis–deafness syndrome (KID syndrome), also known as ichthyosiform erythroderma, corneal involvement, and deafness, presents at birth/infancy and is characterized by progressive corneal opacification, either mild generalized hyperkeratosis or discrete erythematous plaques, and neurosensory deafness.