Ichthyosis lamellaris

Ichthyosis lammellaris, also known as (recessive) lamellar ichthyosis and nonbullous congenital ichthyosis, is a rare inherited skin disorder, affecting less than 1 person in 300,000.

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Presentation

Affected babies are born in a collodion membrane, a shiny waxy outer layer to the skin. This is shed 10-14 days after birth, revealing the main symptom of the disease, extensive scaling of the skin caused by hyperkeratosis. As the child grows, other symptoms manifest themselves, including rapid nail and hair growth. The skin can be put under tension by the scaling, possibly resulting in small ears, inflexible fingers and ectropion, the turning outwards of the eyelids or lips.

With increasing age, the scaling tends to be concentrated around joints in areas such as the groin, the armpits, the inside of the elbow and the neck. The scales often tile the skin and may resemble fish scales.

Genetics

The condition is a recessive genetic disease. It is associated with keratinocyte transglutaminase.

See also

• Ichthyosis
• Bullous congenital ichthyosiform erythroderma