Ichthyosis Vulgaris

What is ichthyosis vulgaris?

Ichthyosis is a skin disease with more than 20 varieties. Ichthyosis vulgaris is the most common and mildest form of ichthyosis. Of those who have some form of ichthyosis, 95% have ichthyosis vulgaris.

The main feature of ichthyosis vulgaris is dry, thick and scaly skin. The condition can begin in childhood, often in the first year of life. Adults can also develop the disease.

Ichthyosis vulgaris is fairly common, affecting one of every 250-300 people (male and female, and of all races).

What causes ichthyosis vulgaris?

Ichthyosis vulgaris is most often caused by one’s genes (inherited). However, some patients may “acquire” ichthyosis vulgaris because of a medical illness or in response to a medication.

In ichthyosis vulgaris, skin cells reproduce at a normal rate, but they don’t separate at the skin’s surface, as they normally do. Also, dead skin cells don’t shed quickly enough, causing a buildup of scales.

Ichthyosis vulgaris in children is usually caused by a gene for the disease that is inherited from one or both parents. This is called inherited ichthyosis vulgaris. Parents don’t have to have the disease to pass the gene onto their children. A child who has inherited the ichthyosis vulgaris gene has a 50% chance of developing the condition.

Another potential cause of ichthyosis vulgaris is a gene mutation (change) that can occur during fetal development. If this happens, the child’s skin lacks enough of a key protein called filaggrin, which helps the body create the outermost layer of skin and shed dead skin cells.

In adults, the disease is called acquired ichthyosis vulgaris, and can be caused by other diseases, including kidney failure, certain cancers, sarcoidosis, leprosy, and human immunodeficiency virus (HIV). On rare occasions, it can be caused by a medication, such as cimetidine (Tagamet®), which treats ulcers and acid reflux, and clofazimine (Lamprene®) which treats leprosy – or by a vitamin, like nicotinic acid (a B vitamin).

The symptoms of ichthyosis vulgaris are the same whether it is inherited or acquired.

What are the symptoms of ichthyosis vulgaris?

Symptoms of ichthyosis vulgaris include:

• Dry, itchy skin
• Thick, rough skin that looks dirty, most noticeably on the palms and soles of the feet
• White, gray or brown scales on the front of the legs, back of the arms, scalp, back or stomach. If scales appear on the face, it’s mostly on the forehead and cheeks. Sometimes the edges of the scales curl, making the skin feel rough.
• Extra lines on the palms of the hands and the soles of the feet. The lines may run deep in severe cases, causing cracks in the skin. If the cracks are deep enough, an infection may develop.
• Rough bumps on the arms, thighs and buttocks (keratosis pilaris). The bumps are often mistaken for acne blemishes.
• Inability to perspire (sweat) adequately. This happens in severe cases and can cause overheating (because perspiration cools down the body).

Many patients don’t realize they have ichthyosis vulgaris because symptoms can be so mild. They simply believe their skin is dry, so they apply moisturizer, which can reduce scaling.

Ichthyosis vulgaris tends to get worse during the winter, when cold and dry air can cause symptoms. In the summer, the disease can virtually disappear because of the warm, moist air.

Sometimes patients notice ichthyosis vulgaris symptoms before they are diagnosed with more serious diseases. On the other hand, ichthyosis vulgaris symptoms sometime appear years after patients are diagnosed with more serious conditions.

How is ichthyosis vulgaris diagnosed?

Ichthyosis vulgaris is often mistaken for simple dry and scaly skin, so it can easily go undiagnosed. If applying moisturizer twice a day doesn’t help, a dermatologist can diagnose ichthyosis vulgaris by examining the skin and distinguishing the condition from other skin diseases.

Before seeing the doctor:

• Find out if any of your blood relatives have similar skin conditions.
• Take note of when the symptoms first appeared.
• Make a list of any other medical or skin conditions you have, and the medications, vitamins and supplements you are taking.

The doctor might perform a biopsy (removing a small portion of the skin for examination under a microscope). Since the disease is inherited in children, the doctor might also order a genetic test.

How is ichthyosis vulgaris treated?

There is no cure for ichthyosis vulgaris, but patients can find relief from the dry, scaly skin. A doctor may suggest you do the following:

• Take baths more than once a day. Hydrating the skin softens the scales.
• Before bathing, apply petroleum jelly or other thick, bland emollients to open sores or deep cracks. Such ointments can ease the burning or stinging that the water might cause and can rid the skin of deep cracks. Adding sea salt to bath water may also reduce burning, stinging and itching.
• Immediately after bathing, apply a liberal amount of moisturizer. Moisturizer can lock in moisture from the bath into the skin. Certain heavy duty moisturizers that include the chemicals lactic acid, salicylic acid and urea can also help the skin shed properly.
• Take oral and/or skin medications the doctor prescribes, including antibiotics to treat skin infections.
• Add a small amount of bleach to bath water if skin infections are frequent. Bleach reduces the number of bacteria on the skin. (Take this step only if directed by a doctor.)
• Rub scales gently with an abrasive sponge while bathing, after the bathwater has softened the scales. This will remove dead skin.
• Treat the disease, or reduce the dose of medication, that caused acquired ichthyosis vulgaris.

What complications are associated with ichthyosis vulgaris?

Complications associated with ichthyosis vulgaris include the following:

• Skin infections
• Children who have the disease may have a higher risk of developing eczema, asthma, hay fever and hives.
• Ichthyosis vulgaris may cause dehydration, blistering, overheating, rapid calorie loss and skin allergies.

Also, a patient may suffer psychologically because of the skin’s appearance.

What is the long-term prognosis (outlook) for people who have ichthyosis vulgaris?

For some children with ichthyosis vulgaris, the scales become less noticeable during puberty. In other cases, symptoms can disappear during childhood and return during the teen years, or even adulthood.

The condition usually improves with age. For the most part, people who have ichthyosis vulgaris live a normal life, although they will probably always have to treat their skin. The disease rarely affects overall health.