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Lamellar ichthyosis
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Lamellar ichthyosis
Lamellar ichthyosis, also known as ichthyosis lamellaris and nonbullous congenital ichthyosis, is a rare inherited skin disorder, affecting around 1 in 600,000 people.
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. The scales often tile the skin and may resemble fish scales.
In medicine, the term collodion baby applies to newborns who appear to have an extra layer of skin (known as a collodion membrane) that has a collodion-like quality. It is a descriptive term, not a specific diagnosis or disorder; as such, it is a syndrome.
The appearance is often described as a shiny film that resembles a layer of Vaseline. The eyelids and mouth may have the appearance of being forced open due to the tightness of the skin. There can also be associated eversion of the eyelids (ectropion).
Collodion babies can have severe medical consequences, mainly because the baby can lose heat and fluid through the abnormal skin. This can lead to hypothermia and dehydration. Strategies to prevent these problems include the use of emollients or nursing the baby in a humidified incubator. There is an increased risk of skin infection and mechanical compression, leading to problems like limb ischemia. There is also a risk of intoxication by cutaneous absorption of topical products; for example, salicylate intoxication (similar to aspirin overdose) due to keratolytics.
The condition is not thought to be painful or in itself distressing to the child. Nursing usually takes place in a neonatal intensive care unit, and good intensive care seems to have improved the prognosis markedly. The collodion membrane should peel off or "shed" 2 to 4 weeks after birth, revealing the underlying skin disorder.
The condition can resemble but is different from harlequin type ichthyosis.
The appearance can be caused by several skin diseases, and it is most often not associated with other birth defects. In most cases, the baby develops an ichthyosis or ichthyosis-like condition or other rare skin disorder.
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Lamellar ichthyosis
Lamellar ichthyosis, also known as ichthyosis lamellaris and nonbullous congenital ichthyosis, is a rare inherited skin disorder, affecting around 1 in 600,000 people.
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. The scales often tile the skin and may resemble fish scales.
In medicine, the term collodion baby applies to newborns who appear to have an extra layer of skin (known as a collodion membrane) that has a collodion-like quality. It is a descriptive term, not a specific diagnosis or disorder; as such, it is a syndrome.
The appearance is often described as a shiny film that resembles a layer of Vaseline. The eyelids and mouth may have the appearance of being forced open due to the tightness of the skin. There can also be associated eversion of the eyelids (ectropion).
Collodion babies can have severe medical consequences, mainly because the baby can lose heat and fluid through the abnormal skin. This can lead to hypothermia and dehydration. Strategies to prevent these problems include the use of emollients or nursing the baby in a humidified incubator. There is an increased risk of skin infection and mechanical compression, leading to problems like limb ischemia. There is also a risk of intoxication by cutaneous absorption of topical products; for example, salicylate intoxication (similar to aspirin overdose) due to keratolytics.
The condition is not thought to be painful or in itself distressing to the child. Nursing usually takes place in a neonatal intensive care unit, and good intensive care seems to have improved the prognosis markedly. The collodion membrane should peel off or "shed" 2 to 4 weeks after birth, revealing the underlying skin disorder.
The condition can resemble but is different from harlequin type ichthyosis.
The appearance can be caused by several skin diseases, and it is most often not associated with other birth defects. In most cases, the baby develops an ichthyosis or ichthyosis-like condition or other rare skin disorder.
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