Kids Health Info

Ichthyosis

  • Ichthyosis means 'fish scale' and is the name of a group of genetic skin diseases that cause dry, scaly, thickened skin. The condition is usually present at birth or in childhood. It is not caused by infection and is not contagious (i.e. it cannot be caught by others). The life span of someone with ichthyosis is normal. There is no known cure for ichthyosis, but there are treatments available which can help to improve the skin's condition.

    Our skin has many important functions including physical protection, temperature regulation and providing a barrier to water and infection. Skin cells are the building blocks of skin and are made up of keratin. 

    In normal skin, new skin cells are formed in the deepest layer at the same time as dead cells are shed from the top layer of the skin. In ichthyosis, either the dead cells are shed too slowly, causing a build up of the top dry layer; or the production of new cells from the lower layers is too slow. The skin barrier is abnormal and allows too much water to be lost from the skin.

    Causes

    Ichythosis is a genetic disorder. It can be passed down from parents to children, or it can be acquired.

    Signs and symptoms

    • The skin may appear normal at birth but then gradually dries out and shrinks and starts to look thick, scaly, dry and cracked.
    • Most signs and symptoms appear by the age of five years.
    • All parts of the body can be affected by ichthyosis, including the face and scalp. However, the bends in the arms and legs are not usually affected.
    • The palms of the hands tend to have a lot of skin.

    Treatment

    People with ichthyosis may need to spend a few hours each day caring for their skin. There are two main sorts of medications in cream form for the treatment of ichthyosis.

    1 - Keratolytics:  

    Keratolytics help to loosen the scales and encourage them to come off and are found in creams which also moisturise the skin.  Unfortunately, they can also be irritating, cause redness, stinging, itching or discomfort. The strength of the cream can be changed if any of these symptoms occur.

    Examples of keratolytics include:

     

    • Lactic acid and salicylic acid 1-5% creams.
    • Ammonium lactate e.g. Lanate cream.
    • Hydroxy-acid creams e.g. Neoceutical brand, Neostrata brand.
    • Urea creams e.g. Urederm, Eulactol, Calmurid.

     

    2 - Retinoids: e.g. Neotigason

    In severe cases of ichthyosis, oral medicine can be helpful to help get rid of the scale, redness and itch. The tablets come from vitamin A. Taking high doses of vitamin A can cause side effects such as dry eyes, lips and nose, nose bleeds, headaches, nausea, high blood cholesterol and birth defects if taken during pregnancy. These medicines can only be prescribed by dermatologists and require strict monitoring with regular check-ups and blood tests.

    A person with ichthyosis can also:

    • rub a pumice stone over wet skin daily to help remove some of the thick crusty skin
    • brush washed hair to remove scales from the scalp
    • have antibiotics prescribed if there is an infection
    • use lanolin creams to help keep their skin moist.

    Care at home

    • Moisturisers help improve the condition of skin dryness. They replace the lost moisture and hold it in the skin. They need to be applied regularly, particularly after showering or bathing. Examples of moisturisers include paraffin, e.g. Dermeze, QV cream; and sorbolene plus glycerin, e.g. Hydraderm.
    • It is a good idea to avoid soap, which can dry the skin out even more. A soap substitute should be used instead. If recurrent infection is a problem, an antiseptic may be used two to three times a week. Examples of soap substitutes are:
      • Oils:  Hamilton's oil, QV oil, Dermaveen oil.
      • Washes: Hamilton's wash, QV wash, Dermaveen wash.
      • Antiseptics: QV "Flare Up" oil, Microsheild T (blue), Oilatum Plus.
    • For the scalp, shampoos containing salicylic acid or tar can reduce the scaling and help to relieve the itch. Examples include Neoceutical shampoo and Ducray 'Kertyol' shampoo.

    Follow-up

    See your doctor if your child's skin suddenly gets worse with pain, cracking or oozing. If you are worried about the treatment, talk to your child's doctor. If your child is taking tablets, don't forget to come back for regular blood tests and prescriptions.

    Key points to remember

    • Most patients will have a mild form of ichthyosis, which does not have a big effect on their general health and wellbeing.
    • Some patients find it uncomfortable or embarrassing.
    • In rare cases, it can be very severe affecting the entire skin surface or causing blisters. In this case, repeated infections, discomfort and sweating problems may occur.
    • Depending on the type of ichthyosis, it may be inherited and therefore passed down to a person's own children.

    More information

    Ichthyosis can be distressing for patients and their families. Patients, particularly children, may suffer from poor self-esteem, teasing or bullying. Meeting with other young people in a similar situation can be helpful.

    • The Ichthyosis Clinic at The Royal Children's Hospital provides the opportunity to meet other patients and families in an informal setting. Genetic counselling can also be arranged to discuss any aspect of the inheritance and risk to future pregnancies. Prenatal testing is available for some types of ichthyosis.
    • There is a support group based in Britain www.ichthyosis.co.uk that may be helpful.
    • Another ichthyosis support group is based in America and is called F.I.R.S.T. www.scalyskin.org
    • www.dermnetnz.org

     

    Developed by the RCH Dermatology Department. First published January 2004. Updated November 2010



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This information is intended to support, not replace, discussion with your doctor or healthcare professionals. The authors of these consumer health information handouts have made a considerable effort to ensure the information is accurate, up to date and easy to understand. The Royal Children's Hospital, Melbourne accepts no responsibility for any inaccuracies, information perceived as misleading, or the success of any treatment regimen detailed in these handouts. Information contained in the handouts is updated regularly and therefore you should always check you are referring to the most recent version of the handout. The onus is on you, the user, to ensure that you have downloaded the most up-to-date version of a consumer health information handout.