Lamellar ichthyosis

Lamellar ichthyosis, also called ichthyosis lamellaris or nonbullous congenital ichthyosis, is a rare inherited skin disorder affecting around 1 in 600,000 people, caused by autosomal recessive mutations and present at birth. Affected newborns are typically born encased in a glossy, waxy outer skin layer called a collodion membrane, which is shed within 10 to 14 days. The lasting feature is widespread scaling of the skin from hyperkeratosis, which tends to concentrate around the joints as the child grows. Both parents must carry one copy of a defective gene for a child to be affected. No cure exists; management relies on regular moisturizing, avoiding overheating, eye drops, and systemic retinoids such as isotretinoin and acitretin, with monitoring for their toxicity, plus psychological support.