Ichthyosis vulgaris

  • Overview
    ichthyosis-vulgaris-arm.jpg
    Ichthyosis vulgaris: The skin cells build up, causing thick flakes that can resemble fish scales.

    Ichthyosis vulgaris: overview

    Ichthyosis (ick-thee-OH-sis) is a group of skin diseases that causes extremely dry, thick, and scaly skin. The skin often looks like it has fish scales.

    There are more than 20 different types of ichthyosis. The most common type is ichthyosis vulgaris (vul-GAR-ris). About 95% of people who develop ichthyosis get this type.

    The other types are rare and include harlequin ichthyosis, lamellar type, and x-linked ichthyosis.

    Of all the types, ichthyosis vulgaris is the mildest. It often begins in childhood.

    Ichthyosis isn’t contagious, so you cannot catch it from someone.

    Children usually develop it when they inherit a gene(s) for the disease from one or both parents. A parent doesn’t have to have the disease to pass on these genes. Because it is passed through the genes, this type is called inherited ichthyosis vulgaris.

    Adults can also develop ichthyosis vulgaris, but this is rare. Adults get acquired ichthyosis vulgaris. It’s called “acquired” because you get it when a disease or medicine that you’re taking causes ichthyosis to develop. Diseases that can trigger this type of ichthyosis include kidney failure, some cancers, and human immunodeficiency virus (HIV).

    Whether ichthyosis vulgaris begins in a child or adult, it can be so mild that is mistaken for extremely dry skin. Many people never realize they have ichthyosis because applying moisturizer keeps their skin free of scale.

    When to see a dermatologist

    If applying moisturizer twice a day fails to get rid of the scaly skin, you should see a dermatologist to find out if you have ichthyosis or another skin condition.

    Image used with permission of Journal of the American Academy of Dermatology: J Am Acad Dermatol. 2006; 55:647-56.

    References
    Fleckman P and DiGiovanna JJ. “The ichthyoses.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:401-424.

    James WD, Berger TG, et al. “Genodermatoses and congenital anomalies.” In: James WD, Berger TG, et al. Andrews' Diseases of the Skin, Clinical Dermatology (tenth edition), Elsevier, Canada, 2006:560-1.

    Richard G and Ringpfeil F. “Ichthyoses, erythrokeratodermas, and related disorders.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:743-9.

    Schwartz RA. “Hereditary and acquired ichthyosis vulgaris.” In: Elston DM, (chief editor) Medscape. Last updated June 22, 2016.


    Ichthyosis vulgaris

    Ichthyosis vulgaris: Overview
    Ichthyosis vulgaris: Signs and symptoms
    Ichthyosis vulgaris: Who gets and causes
    Ichthyosis vulgaris: Diagnosis, treatment, and outcome
    Ichthyosis vulgaris: Tips for managing
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  • Symptoms
    ichthyosis-vulgaris-palm.jpg
    The palms and soles tend not to develop scale, but they can have many lines, as shown here.

    Ichthyosis vulgaris: Signs and symptoms

    This type of ichthyosis often begins in childhood. Most children have normal skin at birth. Between 3 months and 5 years of age is when parents usually notice changes to their child’s skin. Sometimes, these changes begin when the child is younger or older.

    When the changes begin, parents may notice one or more of the following:

    • Dry skin:  A mild case of ichthyosis vulgaris can be mistaken for dry skin.
    • Scales: These tend to be white, gray, or brown. The edges often curl, making the skin feel rough.

      You’ll usually see scales on one or more of these areas — fronts of the legs, backs of the arms, scalp, back, or belly. Scales can develop on other areas, too, but tend to skip the armpits, creases of the elbows, backs of the knees, and the diaper area.
    • Thickened skin: This tends to be most noticeable on the palms and soles. The thickened skin can look dirty.
    • Many lines on the palms and soles: If the ichthyosis is severe, you may see deep cracks on the palms and soles. An infection may develop in the deep cracks.
    • Itchy skin: The itch is often caused by dry skin.
    • Rough bumps on the skin: These bumps can be mistaken for acne and usually develop on the arms, thighs, and buttocks. Many people who do not have ichthyosis get this. The medical name for this condition is keratosis pilaris.
    • Unable to sweat enough: If the ichthyosis is very severe, a child (or adult) may not be able to sweat normally. Being aware of this can prevent the person from overheating.

    Ichthyosis vulgaris in adults

    When ichthyosis vulgaris begins in adults, a disease or medicine is often the cause. While the cause is different in adults, the signs and symptoms are the same as in children.

    Some adults notice changes to their skin before they are diagnosed with a condition like kidney disease, cancer, or human immunodeficiency virus (HIV). Signs and symptoms of ichthyosis can also begin years after the person is diagnosed with a disease like HIV or cancer.

    Ichthyosis vulgaris can change with age

    In some children, the scale becomes more noticeable until the child reaches puberty and then it lessens. It’s also possible for the signs and symptoms to disappear for a while and return during the teenage years.

    If a child had a mild case that was mistaken for dry skin, it may seem that the ichthyosis is just beginning in the teenage years.

    The disease can also return later in life when the person is an adult.

    More obvious (worsens) in winter

    The signs and symptoms tend to worsen in the winter when the air is cold and dry. During hot and humid weather, ichthyosis vulgaris may be barely noticeable or fade completely.

    Watch for related conditions

    Children who develop ichthyosis vulgaris also have a higher risk of developing the following conditions:


    Image used with permission of Journal of the American Academy of Dermatology: J Am Acad Dermatol. 2010; 63:607-41.

    References
    James WD, Berger TG, et al. “Genodermatoses and congenital anomalies.” In: James WD, Berger TG, et al. Andrews' Diseases of the Skin, Clinical Dermatology (tenth edition), Elsevier, Canada, 2006:560-1.

    Patel N, Spencer LA, et al. “Acquired ichthyosis.” J Am Acad Dermatol. 2006;55(4):647-56.

    Schwartz RA. “Hereditary and acquired ichthyosis vulgaris.” In: Elston DM, (chief editor) Medscape. Last updated June 22, 2016.


    Ichthyosis vulgaris

    Ichthyosis vulgaris: Overview
    Ichthyosis vulgaris: Signs and symptoms
    Ichthyosis vulgaris: Who gets and causes
    Ichthyosis vulgaris: Diagnosis, treatment, and outcome
    Ichthyosis vulgaris: Tips for managing
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  • Causes

    Ichthyosis vulgaris: Who gets and causes

    Who gets ichthyosis vulgaris?

    To get ichthyosis vulgaris, one of the following must happen. The person can:

    •  Inherit genes for ichthyosis from one or both parents
    • Develop a gene mutation for ichthyosis while in the womb
    • Get a disease or take a medicine that can trigger ichthyosis

    When caused by genes, the disease appears during childhood — usually between 3 months and 5 years of age. This type of ichthyosis vulgaris is called inherited ichthyosis vulgaris. It often runs in families.

    The other type of ichthyosis vulgaris is very rare. Called acquired ichthyosis vulgaris, this type usually affects adults only. It appears when it is triggered by a disease or medicine.

    People of all races get ichthyosis vulgaris. About the same number of males and females develop this disease.

    What causes ichthyosis vulgaris?

    Inherited ichthyosis vulgaris is caused by genes. The person either inherits the genes for this disease from one or both parents or genes changes while the baby is forming inside the womb. As a result, the skin has less filaggrin (fill-lag-grin) than it needs. The body needs filaggrin to create a healthy outermost layer of skin.

    Without enough filaggrin, the body cannot shed skin cells as it should. The older skin cells build up on the skin’s surface, causing the scale and thickened skin.

    Acquired ichthyosis vulgaris has a different cause. A disease usually triggers it. Diseases that can trigger it include long-term kidney failure, different types of cancer (especially lymphoma), sarcoidosis, and infections like leprosy and HIV.

    Acquired ichthyosis vulgaris can show up before the person has signs of the triggering disease. If this happens, the person should have a complete physical. It’s important to find the cause.

    In very rare cases, a medicine or vitamin can trigger acquired ichthyosis vulgaris. Ones that can do this, include:

    • Cimetidine (an antacid and antihistamine used to treat ulcers and acid reflux)
    • Clofazimine (a drug used to treat leprosy)
    • Nicotinic acid, a B vitamin

    References
    Brown SJ, McLean WH. “One remarkable molecule: filaggrin.” J Invest Dermatol. 2012 Mar;132(3 Pt 2):751-62.

    James WD, Berger TG, et al. “Genodermatoses and congenital anomalies.” In: James WD, Berger TG, et al. Andrews' Diseases of the Skin, Clinical Dermatology (tenth edition), Elsevier, Canada, 2006:560-1.

    Richard G and Ringpfeil F. “Ichthyoses, erythrokeratodermas, and related disorders.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:743-9.

    Schwartz RA. “Hereditary and acquired ichthyosis vulgaris.” In: Elston DM, (chief editor) Medscape. Last updated June 22, 2016.


    Ichthyosis vulgaris

    Ichthyosis vulgaris: Overview
    Ichthyosis vulgaris: Signs and symptoms
    Ichthyosis vulgaris: Who gets and causes
    Ichthyosis vulgaris: Diagnosis, treatment, and outcome
    Ichthyosis vulgaris: Tips for managing
  • Treatment
    ichthyosis-vulgaris-treatment.jpg
    Soaking, moisturizing, and removing the scale can also get rid of the rough bumps on the skin, known as keratosis pilaris.

    Ichthyosis vulgaris: Diagnosis and treatment

    How do dermatologists diagnose ichthyosis vulgaris?

    A dermatologist can often diagnose ichthyosis vulgaris by looking at a patient’s skin.

    To make this diagnosis, your dermatologist will also ask a few questions. Before your appointment, it can be helpful to make sure you have answers to the following questions:

    • Do blood relatives in your (your child’s) family have a similar skin condition?
    • How old were you (your child) when the skin condition began?
    • Do you (your child) have any other skin conditions?
    • Do you (your child) have any other medical conditions?
    • What medicines and supplements do you (or your child) take?

    Sometimes, ichthyosis vulgaris looks like another skin condition. If this happens, your dermatologist can remove a small amount of skin so that it can be looked at under a microscope. This is called a skin biopsy. Your dermatologist can quickly and easily remove the skin during an appointment.

    How do dermatologists treat ichthyosis vulgaris?

    There is no cure for inherited ichthyosis vulgaris. Treatment focuses on reducing the scale and dry skin. To do this, a treatment plan may require you to:

    • Take baths as often as directed: Soaking helps hydrate your skin and soften the scale.

      If you have open sores, your dermatologist may recommend placing petroleum jelly or another product on these before getting into the water. This can reduce the burning and stinging caused by the water.

      Some patients say that adding sea salt (or table salt) to the water reduces the burning and stinging. Adding salt may also reduce the itch.
    • Reduce the scale during your baths: Soaking in water softens the scale. Your dermatologist may recommend that you reduce the scale while it’s softer by gently rubbing the scale with an abrasive sponge, buff puff, or pumice stone.
    • Apply moisturizer to damp skin within 2 minutes of bathing: Moisturizer can seal water from a bath or shower into your skin. Your dermatologist may recommend a moisturizer that contains an active ingredient like urea, alpha hydroxyl acid, or lactic acid. These and other active ingredients can also help reduce scale.
    • Apply petroleum jelly to the deep cracks: This can help get rid of the deep cracks.
    • Treat a skin infection: If you develop a skin infection, your dermatologist will treat it with either medicine you take or apply to your skin.

      If you get frequent skin infections, your dermatologist may recommend adding a small amount of bleach to your bath. This can help reduce the amount of bacteria on your skin. You should do this only if your dermatologist recommends it.

    If the ichthyosis is severe and fails to improve with baths, moisturizer, and scale remover, your dermatologist can prescribe medicine.

    Some people only need to treat their skin in the winter: If the ichthyosis is mild, you may need to treat it only during the winter. In humid climates, most ichthyosis improves. A mild case can clear completely, returning in the winter when the air is dry.

    What is the outlook for someone who has ichthyosis vulgaris?

    For people who have the inherited type, the outlook is excellent. Most have a normal lifespan. Ichthyosis vulgaris also can become less serious with age. Most people, however, need to continue treating their skin for life.

    To improve acquired ichthyosis vulgaris, you must also treat the disease that triggered the ichthyosis. If the disease can be cured, the ichthyosis may go away. If a medicine triggered the ichthyosis, reducing the dose often gets rid of the ichthyosis.

    References
    Fleckman P and DiGiovanna JJ. “The ichthyoses.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:401-424.

    James WD, Berger TG, et al. “Genodermatoses and congenital anomalies.” In: James WD, Berger TG, et al. Andrews' Diseases of the Skin, Clinical Dermatology (tenth edition), Elsevier, Canada, 2006:560-1.

    Patel N, Spencer LA, et al. “Acquired ichthyosis.” J Am Acad Dermatol. 2006;55(4):647-56.

    Richard G and Ringpfeil F. “Ichthyoses, erythrokeratodermas, and related disorders.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:743-9.

    Schwartz RA. “Hereditary and Acquired Ichthyosis Vulgaris.” Medscape. Last accessed July 15, 2016.


    Ichthyosis vulgaris

    Ichthyosis vulgaris: Overview
    Ichthyosis vulgaris: Signs and symptoms
    Ichthyosis vulgaris: Who gets and causes
    Ichthyosis vulgaris: Diagnosis, treatment, and outcome
    Ichthyosis vulgaris: Tips for managing
    Save
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  • Tips
    ichthyosis-vulgaris-tips.jpg
    Girl applying moisturizer: Applying moisturizer helps keep the skin free of scale.

    Ichthyosis vulgaris: Tips for managing

    To help their patients with this long-term skin condition get the best results from treatment, dermatologists often share these tips:

    1. Continue to follow your treatment plan. Continuing to take baths, moisturize, and reduce scale can keep ichthyosis vulgaris under control.

    2. Remove scale gently. If you develop scale, it can be tempting to scrub the scale to remove it as quickly as possible. Scrubbing can make the skin raw, so you want to remove scale gently.

    By working these tips into their daily routine, many people find that they can manage ichthyosis vulgaris effectively.

    Reference
    Fleckman P and DiGiovanna JJ. “The ichthyoses.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:401-424.


    Ichthyosis vulgaris

    Ichthyosis vulgaris: Overview
    Ichthyosis vulgaris: Signs and symptoms
    Ichthyosis vulgaris: Who gets and causes
    Ichthyosis vulgaris: Diagnosis, treatment, and outcome
    Ichthyosis vulgaris: Tips for managing

     


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