Ichthyosis lamellar, autosomal dominant


Type of disease: Rare conditions

Ichthyosis lamellar, autosomal dominant is a very rare disorder where an infant is born covered with a transparent membrane which sheds to reveal red scaly skin patches of varying sizes. They are called “collodion babies” because of the tight shiny membrane resembling plastic wrap, which they are born encased in. Collodion baby is not a disease entity but is the first expression of some forms of ichthyosis. This membrane is usually shed during the first few weeks of life, but remaining effects include scaly skin and outward turning eyelids and lips. People with lamellar ichthyosis typically have large, dark, plate-like scales covering their skin. Affected individuals may also have hair loss (alopecia), a decreased ability to sweat (hypohidrosis), and a thickening of the skin on the palms of the hands and soles of the feet (keratoderma). Less frequently, affected individuals have reddened skin (erythema) and joint deformities (contractures). Infants with lamellar ichthyosis may develop infections, an excessive loss of fluids (dehydration), and respiratory problems.

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