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Ichthyosis Overview

Skin Problems Image Gallery What's the cause of this family of skin disorders, which are marked by deep, cracked skin? See more pictures of skin problems.
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At age 63, Mary had gotten used to the staring and pointing, but not the pain and humiliation of living with crusty, dry skin that looks like fish scales. She's been refused service at a restaurant and asked to leave a public swimming pool. Worst of all, when she was just a young girl attending a county fair in Newton, Kan., a carnival owner offered Mary's mother money -- if only he could take Mary on the road as part of a freak show [source: FIRST].

Mary has lamellar ichthyosis, one of the 28 identified types of a family of skin disorders known as ichthyosis. Like most ichthyosis disorders, it is relatively rare, occurring in one of 200,000 births [source: FIRST]. The skin becomes thick and dry, then darkens and takes on a diamond-shaped pattern like the scales of a fish [source: FIRST]. In fact, the name for this group of skin disorders is derived from the Greek word for fish: "ichthys" [source: Skin Sight].

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If not hydrated by a series of prescription and over-the-counter medicated lotions, lamellar ichthyosis will cause the skin to crack and bleed. Because the skin regenerates constantly -- much faster than the average person's -- but does not shed dead skin cells as it normally would, a thick build-up of skin occurs. Those with the condition can spend several hours scrubbing their skin each week. Despite all that exfoliating, they can continually lose large flakes of skin because it regenerates at such a rapid rate. And, because the damaged skin can inhibit the body's ability to sweat, patients are often in danger of overheating [source: FIRST].

Even so, Mary's one of the fortunate ones. At its most severe, the scaling caused by ichthyosis can cause joint damage, hearing loss, limited movement and even death. Ichthyosis can also be disfiguring, creating fissures so deep they penetrate to the muscle and tighten the skin of the face so the eyelids turn outward [source: Project SOS].

Clearly, ichthyosis is more than skin-deep. There is no cure, and the consequences of leaving this condition's symptoms untreated could prove fatal -- especially for infants born with harlequin ichthyosis. We'll explain more about this most serious type on the next page.

 

In the United States, ichthyosis affects more than 1 million people of all races and both sexes [source: FIRST]. In its mildest form -- xeroderma -- ichthyosis is simply dry skin that can easily be treated by upping your daily dose of water and applying over-the-counter lotions. But those who inherit more serious forms of the disorder are certain to experience more severe symptoms.

If at least one parent has ichthyosis, there's a 50-50 chance this dominant trait will be passed on to the next generation [source: Skin Sight]. There's good news for those with ichthyosis who once worried about passing on their malady through close contact: It simply isn't contagious. But, you can still acquire it as a side effect of diseases such as AIDS or lymphoma [source: Hershey Medical Center College of Medicine]. However, this happens so rarely that the worldwide prevalence of acquired ichthyosis remains unknown [source: Schwartz].

Most people with inherited ichthyosis are diagnosed with ichthyosis vulgaris [source: Mayo Clinic]. Sometimes this skin condition can be seen at birth, but often it becomes apparent during the toddler or preschool years. What signs should you look for? As the skin fails to shed, it accumulates to form thick, dry scales that are white, gray or dark brown in color. It can also cause deep cracks on the soles or palms that can make it painful to do even the most ordinary activities [source: Mayo Clinic].

Other, less common forms of ichthyosis include lamellar ichthyosis, as discussed on the previous page, and X-linked ichthyosis, which affects only males and causes brown scales on the back, arms and knees [source: Mayo Clinic].

Fortunately, harlequin ichthyosis -- the most severe variation in this family of skin disorders -- is very rare, occurring in only one of every 300,000 births [source: Dolunay]. But it's almost always fatal. Almost. In the mid-1980s, when a San Diego, Calif., mother gave birth to a baby with harlequin ichthyosis, no one expected him to live. The infant, Ryan Gonzalez, had eyelids that were turned inside out; his nose, mouth, ears, fingers and toes were disfigured; and the plates of armor-like skin covering his entire body were separated by deep, wide cracks. This meant his skin, although abnormally thick in places, wasn't a protective barrier against dehydration or infection [source: 10News]. For Gonzalez -- and others with ichthyosis -- the first challenge to living with the condition is to keep the skin moist. What relatively common prescription cream helped him survive? We'll tell you on the next page.

Allowing ichthyosis-affected skin to dry out can be deadly. If deep cracks and fissures form, they allow moisture to escape unchecked and germs to penetrate freely. So people with ichthyosis often take several baths a day, then apply a cocktail of lotions and creams to their still-damp skin [source: Mayo Clinic]. This helps seal in moisture and stave off dryness.

A few herbal remedies, such as the application of comfrey or plantain, are thought to help damaged skin cells re-generate [source: altMD]. But doctors often go the prescription route, advising patients to use lotions that contain skin-shedding chemicals, such as glycolic acid or lactic acid, which help the skin slough its dead cells [sources: The New York Times, Mayo Clinic].

In Gonzalez's case, he was one of the first to use a vitamin A-powered prescription acne medication to fight ichthyosis symptoms. The medicine works by slowing the production of skin cells. It worked for Gonzalez -- as a teenager in 2004, he was surviving and training as a triathlete, despite the toll the condition had taken on his body. Unfortunately, the drug comes with a price: hair loss, and swelling of the lips and eyes [source: Mayo Clinic].

For people with ichthyosis, the build-up of skin is another problem, one that often requires manual "de-scaling." This means using a loofah, pumice stone or other abrasive tool to remove layers of thickened skin.

The best treatment, however, may be to get to the root of the problem. Enzyme therapy research has initially been promising, especially for those with lamellar ichthyosis. The hope is to develop liposome-enriched creams. The idea is that, by replacing missing reparative enzymes, skin will begin to regenerate and shed in a more typical manner [source: Traupe].

Learn more about common -- and not so common -- skin disorders by following the links on the next page.

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Sources

  • 10News.com. "Man Survives Rare Skin-Shedding Disease." Nov. 15, 2004. (June 3, 2010)http://www.10news.com/health/3919722/detail.html
  • AltMD.com. "Herbal Remedies for Ichthyosis." AltMD.com. (June 3, 2010).http://www.altmd.com/Articles/Herbal-Remedies-for-Ichthyosis
  • Bale, Sherri. "Genetic Testing for Ichthyoses." ScalySkin.org. 2004. (June 3, 2010)http://www.scalyskin.org/content.cfm?ContentID=194&ColumnID=4
  • Foundation for Ichthyosis and Related Skin Types (FIRST). "About Ichthyosis: Facts and Figures." ScalySkin.org. (June 3, 2010)http://www.scalyskin.org/column.cfm?ColumnID=11
  • Foundation for Ichthyosis and Related Skin Types (FIRST). "About Ichthyosis: Lamellar Ichthyosis." ScalySkin.org. (June 3, 2010)http://www.scalyskin.org/content.cfm?ContentID=91&ColumnID=14
  • Foundation for Ichthyosis and Related Skin Types (FIRST). "Ichthyosis Through the Decades." ScalySkin.org. (June 3, 2010)http://www.scalyskin.org/content.cfm?ContentID=295&ColumnID=34
  • Gurses, Dolunay, et al. "A Case of Harlequin Fetus with Psoriasis in his Family." The Internet Journal of Dermatology. 2001. (June 3, 2010)http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijd/vol1n1/harlequin.xml
  • Hershey Medical Center College of Medicine. "Ichthyosis/Ichthyosis Vulgaris." PennState. (June 3, 2010).http://www.hmc.psu.edu/healthinfo/i/ichthyosis.htm
  • Mayo Clinic. "Ichthyosis Vulgaris." May 11, 2010. (June 3, 2010) MayoClinic.com.http://www.mayoclinic.com/health/ichthyosis-vulgaris/DS00734
  • Prendiville, Julie. "Harlequin Ichthyosis: Differential Diagnoses and Workup." WebMD.com. June 4, 2010. (June 7, 2010).http://emedicine.medscape.com/article/1111503-diagnosis
  • Project Save Our Skin. "About Ichthyosis." ProjectSaveOurSkin.org. (June 3, 2010)http://www.projectsaveourskin.org/about_ichthyosis.htm
  • RxList.com. "Accutane." RxList.com. (June 3, 2010)http://www.rxlist.com/accutane-drug.htm
  • Schwartz, Robert. "Ichthyosis Vulgaris, Hereditary and Acquired." eMedicine from WebMD.com. July 21, 2009. (June 3, 2010)http://emedicine.medscape.com/article/1112753-overview
  • SkinSight.com "Scaly Skin." SkinSight.com. (June 3, 2010)http://www.skinsight.com/adult/ichthyosisVulgaris.htm
  • The New York Times. "Ichthyosis Vulgaris." NYTimes.com. April 16, 2007. (June 3, 2010)http://health.nytimes.com/health/guides/disease/ichthyosis-vulgaris/overview.html
  • Traupe, Heiko. "Liposomal Packing of Recombinant Transglutaminase-1 as an Essential Step Toward Enzyme Replacement Therapy of Transgultaminase-1 Deficient Lamellar Ichthyosis." 2007. (June 3, 2010)http://www.scalyskin.org/content.cfm?ContentID=340&ColumnID=4

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