Bullous congenital ichthyosiform erythroderma (BCIE)

The term is actually a misnomer because Bullous congenital ichthyosiform erythroderma is not a true form of erythroderma, although it does resemble erythroderma clinically.
Bullous congenital ichthyosiform erythroderma is a rare autosomal dominant disorder of keratinization caused by a genetic defect of the epidermal keratins, K1 and K10, which leads to impairment in the tonofilament network of differentiating epidermal cells. The disorder presents at birth with generalized erythema, blistering, and erosions, followed in time by hyperkeratosis. Erythroderma or exfoliative dermatitis describes generalized redness of the skin, usually more than 90 percent of the body surface area, with variable degrees of scaling caused by underlying dermatoses, drug reactions, malignancies, systemic diseases, infections and idiopathic disorders.