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Last updated August 7, 2008 |
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Last updated August 7, 2008 |
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Show N TellSo you have questions about ichthyosis, like: Read on to find out! What is Ichthyosis?The Ichthyoses are a family of skin diseases; the characteristic that they each share is that they cause the skin to build up and scale (some medical journals and dictionaries still refer to ichthyosis as "fish skin disease’). The degree of scaling can vary; some people who have ichthyosis just have dry skin. They can pretty easily care for their skin with drugstore lotions, and unless they told you they had trouble with dry skin, you probably would never know that had it. As you might guess, this type of ichthyosis is fairly common. However, most forms of ichthyosis are much more severe (also very rare). The scaling can be very heavy—you definitely notice right away, just passing someone on the street, that something is wrong with their skin. However, it’s much more than a cosmetic problem for the person who has ichthyosis—the scaling can be very painful, as it can restrict the body’s range of movement, pull so tightly around the face that the eyelids turn outward, cause deep cracks or fissures at the joints, adversely affect hearing, and more. People who have ichthyosis will probably try their best to hide these problems from you. They don’t want you to pity them, and they don’t want their ichthyosis to stop them from doing everything in life that everyone else can do. However, living with ichthyosis can be very tough.
Dermatologists have recognized more than 25 types of ichthyosis (see the list below), however, there are just a handful of main types: Lamellar Ichthyosis, Congenital Ichthyosiform Erythroderma (CIE), Epidermolytic Hyperkeratosis (EH or EHK), X-linked Ichthyosis and Ichthyosis Vulgaris. With the exception of Ichthyosis Vulgaris, Ichthyosis is a very rare disease.
The Ichthyoses and Related Skin Types:
What Does It Look Like?Check out our photo gallery. How Does This Happen?Your skin is alive; it is your body’s largest organ! With "normal" skin, the skin is constantly renewing itself, dying, and shedding (the average skin cell has a lifespan of 14 days). Most of the time you probably don’t even notice that this is going on. When your skin sheds, it’s practically invisible (unless you’re looking at it under a microscope!) because it’s shedding in pieces that are so small. With ichthyosis, the skin doesn’t follow the life cycle that it’s supposed to, and builds up. In CIE and EH, the cells reproduce too fast, faster than they can shed, and build up. In Lamellar Ichthyosis, X-linked Ichthyosis and Ichthyosis Vulgaris, the skin cells reproduce at a normal rate, but they don’t shed as quickly as they should, and again produce a build-up.
Why Did This Happen To You?
That’s all most people need or want to know. Genetics is a complex subject, including the genetics of ichthyosis. For more information about the genetics of ichthyosis, check out the Library. Also, there are some rare, acquired (non-genetic) forms of ichthyosis, but these are also not contagious. These are typically associated with a variety of other conditions, including cancer and AIDS.
Incidence RatesIt’s no wonder that with so many different kinds of recognized ichthyosis, sometimes it’s hard to accurately diagnose certain individuals. Because many people with milder forms of ichthyosis go undiagnosed (they may figure they just have dry skin and don’t seek special treatment from a dermatologist), and others may be misdiagnosed, exact incidence rates are difficult to calculate. However, the table below shows commonly accepted estimates of incidence rates:
Source: Foundation for Ichthyosis and Related Skin Types
This certainly qualifies ichthyosis as a rare disease. A rare or "orphan" disease affects fewer than 200,000 people in the United States. However, we're hardly alone in being affected by a rare disease. There are more than 5,000 rare disorders that, taken together, affect approximately 20 million Americans. One in every 12 individuals in this country has received a diagnosis of a rare disease.
Compare this with the incidence rates of things you may be more familiar with:
We're not alone in having skin problems either; some notable celebrities have skin problems too!
What Is It Like to Have Ichthyosis?One of the things your skin normally does for you is regulate your internal temperature. People with ichthyosis are often more sensitive to temperature changes, both hot and cold. However, overheating poses the biggest problem. Hot weather, vigorous physical exercise, anything that would normally cause you to sweat and be hot, poses an issue for people with ichthyosis (many of whom can't perspire, or only sweat a small bit in certain places...not enough to naturally cool themselves down). So people with ichthyosis go to great lengths to stay active and avoid heat exhaustion (or worse!).
There currently is no cure for ichthyosis; only treatments. Caring for ichthyosis is very labor-intensive. It means spending lots of hours every week bathing, scrubbing the skin in an effort to shed some of the scales, putting on creams to help moisturize and exfoliate....it's a never-ending battle, where you can never quite seem to get the upper hand. Caring for ichthyosis is as much about looking good as feeling good. For people with severe forms of ichthyosis, the goal (which is tough to achieve), is two-fold: make the skin look "normal" enough that people on the street don't stare, call you names, or always ask you what's wrong with you, and secondly to make the skin feel good, so that your skin doesn't make it painful to move, impair your hearing or effect your eyesight. For more details on what people do to care for their skin, see Helpful Hints.
You Can Help!Even without committing very much time or money. Check out How You Can Help for more information.
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Ichthyosis
is caused by a genetic defect (either by
spontaneous mutation of a gene or through family
inheritance). The symptoms may or may not be
apparent at birth, but the genetic defect was
present at conception. You cannot
"catch" ichthyosis. You cannot do
anything wrong to develop ichthyosis. It’s not
your parents’ fault either. You were just born
with it.