Ichthyosis vulgaris
Introduction
Ichthyosis (ik-thee-ow-suhs) refers broadly to a group of skin diseases where patients have dry, thickened and scaly skin. Ichthyosis vulgaris (vuhl-gaa-ruhs) is the most common type of ichthyosis. The word ichthyosis means 'fish scale' in Greek, which is a good description of what the skin looks like in this condition.
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Dry, thickened, scaly skin on legs in a child with ichthyosis vulgaris
Who gets it?
Ichthyosis vulgaris affects 1 in 250 children, and it affects boys and girls equally. Often children develop signs of ichthyosis vulgaris in their first year of life, with the majority of cases appearing before the age of 5.
What causes it?
Ichthyosis vulgaris is caused by a “manufacturing error” or a genetic defect that happens before birth when your baby is developing in the womb in the gene that produces a protein calle filaggrin. Filaggrin is important for keeping the outer layer of skin healthy and helps the skin hold onto moisture. Therefore, a problem with this gene can result in dry and flaky skin.
Only one parent needs to have the abnormal gene for this disorder to be passed on to their child giving it a 50% chance of getting ichthyosis vulgaris.
An error in the same gene is also responsible for children developing atopic dermatitis or eczema. So children with ichthyosis vulgaris also commonly develop eczema. Most babies with ichthyosis vulgaris have no other medical problems and are healthy and well.
What does it look and feel like?
Looks like…
Your baby may have normal skin at birth and signs may not develop until they are school age.
The skin is dry and thickened.
White flakes can shed from the skin.
It tends to affect the abdomen, arms and legs, and not skinfolds such as the armpit and neck.
An increased number of skin lines on the palms and soles is common - known as hyperlinear palms.
Little bumps may occur on the face, upper arms, and thighs - known as keratosis pilaris.
There may be cracks on the heels and palms – this can increase the risk of infection.
Feels like…
Your child may become itchy due to the dryness or from having eczema as well.
Your child's skin will feel rough.
What tests might be needed?
A dermatologist will be able to diagnose ichthyosis vulgaris by looking at your child’s skin and asking a few questions about his/her skin. If the diagnosis is unclear, they may need to take a small sample of skin called a skin biopsy to confirm the diagnosis.
What treatments are available?
What can I do?
Bathing: Your child should only bathe when necessary - in young children this can be limited to 2-3 times per week. Baths should be quick and water temperature as cool as possible.
Skin moisturisers should then be applied within 5 minutes of bathing, as well as regularly throughout the day. Thick oily moisturisers are preferrable to light cream based moisturisers.
Exfoliation with pumice stone or with creams must be undertaken with care.
Medical therapies
Creams containing urea, alpha-hydroxy acid or lactic acid can be used but must be given under medical supervision in young children due to the potential of irritation and toxicity.
Antibiotics may be prescribed if your child gets an infection, particularly if they have eczema.
What happens after treatment?
Ichthyosis vulgaris is unlikely to completely resolve, but it usually improves with age. It is generally well managed with regular moisturisers life-long.
What support is available?
Your GP or your dermatologist will be able to provide medical advice to help manage your child’s symptoms.
Authors/Reviewers:
Author: Dr Samantha Ting
Paediatric Reviewer: Dr Mayuri Sivagnanam
Editor in Chief: Dr Tevi Wain
