Affected babies are born in a collodion membrane – a shiny, waxy-appearing outer layer on 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.

With increasing age, the scaling tends to become concentrated around joints in areas such as the groin, the armpits, the inside of the elbow, and the neck.

[3] There is an increased risk of skin infection and mechanical compression, leading to problems like limb ischemia.

[1] The collodion membrane should peel off or "shed" 2 to 4 weeks after birth, revealing the underlying skin disorder.

[2] In most cases, the baby develops an ichthyosis or ichthyosis-like condition or other rare skin disorder.

Overheating: The scaling of the skin prevents normal sweating so hot weather and/or vigorous exercise can cause problems.