“Jenna” had a problem. She had visited more than 10 doctors, but no one could identify the cause of the cracked and peeling skin on her legs. They were incredibly painful, especially at night, and made her skin look like it was made of fish scales instead of human flesh.
Finally, a doctor diagnosed ichthyosis, a rare skin disorder that leaves skin so dry and flaky it literally looks like it belongs to another species. In fact, the word comes from the ancient Greek root ichthys, meaning, unsurprisingly, fish. While the severity of ichthyosis can vary from individual to individual, Jenna’s case was obviously quite severe.
“Often it was just agony,” says Jenna, 28. “My legs were so sore and when I used to wrap them in bandages at night, the bandages would stick to my legs, making it excruciatingly painful to take them off.
“Whenever I went to a club, I prayed that the lights would stay off, as I was too embarrassed that anyone would see my legs. They looked absolutely horrible, and I would keep them completely hidden during the day just because I felt so embarrassed. “
Finally, a doctor diagnosed ichthyosis, a rare skin disorder that leaves skin so dry and flaky it literally looks like it belongs to another species. In fact, the word comes from the ancient Greek root ichthys, meaning, unsurprisingly, fish. While the severity of ichthyosis can vary from individual to individual, Jenna’s case was obviously quite severe.
What is ichthyosis?
In people with normal skin, the outer layer, or the epidermis, contains cells that are constantly dividing and then shed. People with ichthyosis, however, have skin cells that reproduce at an alarming rate much faster than they can shed, or scales that don’t shed at all, causing a buildup of skin.
The result of this usually genetic skin condition is that cells build up on top of each other, creating a thick, scaly appearance. Sometimes, in severe cases like Jenna’s, the skin becomes almost unbearably dry and cracked, and can even restrict movement. Usually the legs are affected, although sometimes the hands and arms are also affected.
The problem is that this ailment is relatively rare, many doctors and even some dermatologists may not recognize it for what it is, giving the wrong treatment or no treatment at all.
There are several main types of ichthyosis, including:
* Hereditary ichthyosis vulgaris. The most common type, affecting around 95 percent of people with ichthyosis. The skin may appear normal when a child is born, but symptoms usually appear no later than age five. All parts of the body can be affected and it is often associated with atopic dermatitis. It is estimated that at least one in 300 people in the United States has this condition to some degree, and race and gender are not important. A child of a parent with the disorder has a one in two chance of having it as well.
* Acquired ichthyosis. As its name suggests, this type of disorder is acquired and usually appears in adulthood. It is very rare and is often attributed to taking certain medications or because the patient has an underlying medical or other condition, such as HIV/AIDS, leprosy, certain cancers, or glandular problems. Very poor nutrition can also play a role in a person getting ichthyosis.
* Harlequin ichthyosis. A congenital condition in which a baby is born with thick skin all over the body, which eventually cracks, causing deep, painful fissures. Incredibly rare, the chances of having it are only one in a million as both parents must be carriers. Most children born with this condition die shortly after birth due to fluid loss, if they survive the life expectancy is not very high.
Symptoms and signs
It can be difficult for doctors to diagnose ichthyosis, although it is relatively common, as mild forms are easy to confuse with other skin conditions. However, because conditions such as eczema are often treated with steroids, to which ichthyosis does not respond (see below), getting the correct diagnosis is important.
Here are some signs to watch for if you suspect you or your children have ichthyosis vulgaris, the most common type:
* Scaled on the front of the legs
* Scale on the back of the arm
* Peeling on the back or peeling
* Peeling of the cheeks and forehead, which is more prominent in younger children
Keep in mind that there are also certain areas of the body that are not affected by the condition, so if you have itchy, flaky skin in those places, there’s an excellent chance it’s something else. These areas are: the tummy, the face, the front of the neck, and the creases at the front of the knees and elbows (which are often the main places for eczema).
treatment options
While ichthyosis vulgaris may improve with age, ongoing therapy is often required to keep the scaling under control and make the patient more comfortable with themselves. Usually a two-pronged approach is taken, one an emollient or moisturizer to hydrate the skin, the other an ointment to prevent moisture from evaporating and further drying the skin.
Alpha-hydroxy acids are typically used to moisturize, while other solutions are put in to remove hardened scales and reveal smoother skin. Salicylic acid is commonly used, which you would use would depend on your doctor’s advice. Steroids do not usually help, although topical retinoids may be helpful.
In Jenna’s case, she was advised to apply both a moisturizer and ointment several times a day, and to wrap her legs in cellophane every night once they were creamy enough. Within a few months, her condition improved to the point where her scales were no longer visible. “They have literally given me my life back,” she says.
The information in the article is not intended to be a substitute for the medical expertise and advice of your health care provider. We encourage you to discuss any decisions about treatment or care with an appropriate health care provider.