Ichthyosis

Definition:
Ichthyosis (plural ichthyoses) is a heterogeneous family of at least 28, generalized, mostly genetic skin disorders. All types of ichthyosis have dry, thickened, scaly or flaky skin. In many types there is cracked skin, which is said to resemble the scales on a fish; the word ichthyosis comes from the Ancient Greek (ichthys), meaning “fish.” The severity of symptoms can vary enormously, from the mildest types such as ichthyosis vulgaris which may be mistaken for normal dry skin up to life-threatening conditions such as harlequin type ichthyosis. The most common type of ichthyosis is ichthyosis vulgaris, accounting for more than 95% of cases

Ichthyosis  is an inherited skin disorder in which dead skin cells accumulate in thick, dry scales on your skin’s surface. The scales of ichthyosis  can be present at birth, but usually first appear during early childhood. Sometimes ichthyosis  disappears entirely for most of the adult years, only to return later.

Types:
There are many types of Ichthyosis and an exact diagnosis may be difficult. Types of Ichthyosis are classified by their appearance and their genetic cause. Ichthyosis caused by the same gene can vary considerably in severity and symptoms. Some Ichthyosis don’t appear to fit exactly into any one type. Also different genes can produce Ichthyosis with similar symptoms. The most common or well-known types are as follows:

Genetic ichthyosisIchthyosis vulgaris
*X-linked ichthyosis
*Congenital ichthyosiform erythroderma (nbCIE)
*Epidermolytic hyperkeratosis (bullous ichthyosis, bCIE)
*Harlequin type ichthyosis
*Ichthyosis bullosa of Siemens
*Ichthyosis hystrix
*Ichthyosis lamellaris (lamellar ichthyosis)

Ichthyosis with additional characteristics:
*Carvajal syndrome
*CHILD Syndrome
*Conradi-Hünermann syndrome
*Darier’s disease
*Erythrokeratodermia variabilis (Erythrokeratodermia figurata variabilis, Mendes da Costa type erythrokeratodermia)
*IFAP syndrome (Ichthyosis follicularis)
*Keratitis-ichthyosis-deafness syndrome
*Netherton syndrome
*Neutral lipid storage disease (Dorfman-Chanarin syndrome)
*Refsum’s disease
*Rud syndrome
*Senter syndrome
*Sjögren-Larsson syndrome
*Tay syndrome (IBIDS syndrome, Trichothiodystrophy)

Non-genetic ichthyosisIchthyosis
*acquisita

Most cases of ichthyosis  are mild, but some are severe. Sometimes other skin diseases are associated with ichthyosis vulgaris, such as the rash-producing atopic dermatitis. No cure has been found for ichthyosis , and treatments focus on controlling the condition.

 

Symptoms:
In ichthyosis vulgaris, the skin cells are produced at the standard rate, but don’t separate normally as they reach the skin’s surface. This means skin cells aren’t shed as quickly as they should be, leading to a build-up of cells, which appear as scales. Just one or a few areas are usually affected, often the legs or lower body.
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The face isn’t usually affected but, when it is, the scaling is usually limited to the forehead and cheeks. The scales are typically fine and white.

Most babies with ichthyosis vulgaris don’t have any sign of the condition when they’re newly born. But within the first year, skin abnormalities begin to develop.

The dry, scaly skin may then become a chronic, lifelong problem, although it can vary dramatically with age, weather and other factors. It often improves in the summer, for example, and also tends to get better with age.

Causes:
Ichthyosis vulgaris is characterized by chronic, excessive buildup of the protein in the upper layer of the skin (keratin). This buildup is a result of your skin’s natural shedding process being slowed or inhibited.

Ichthyosis vulgaris is most often caused by a genetic mutation, inherited in an autosomal dominant pattern. That means a child has to inherit only one copy of the affected gene to develop the disease. Children with the inherited form of the disorder usually have normal skin at birth, but develop scaling and roughness during the first few years of life. At times, ichthyosis vulgaris may disappear during the adult years, only to return later.

Ichthyosis not caused by genetic abnormalities, referred to as acquired ichthyosis, is rare. This type usually shows up in adulthood. It’s usually associated with other diseases, such as cancer, thyroid disease or chronic renal failure.

Complications:
Ichthyosis can affect more than the appearance of your skin; it can affect your skin’s ability to function normally. As a result, some people with ichthyosis may experience:

*Overheating. In rare cases, ichthyosis interferes with sweating. Skin thickness and scales prevent sweat from reaching the surface of your skin, which inhibits cooling.

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*Secondary infection. Skin splitting and cracking may lead to infections, either on your skin or a wider infection in your body.

 

Diagnosis:
A physician often can diagnose ichthyosis by looking at the skin. A family history is very useful. In some cases, a skin biopsy is done to help to confirm the diagnosis. In a biopsy, a small piece of skin is removed and examined under a microscope. In some instances, genetic testing may be helpful in making a diagnosis. Diabetes has not been linked to acquired ichthyosis or ichthyosis vulgaris.

Ichthyosis is not more or less common in any ethnic group. As of now, there is no way to prevent ichthyosis since it is often of a genetic nature.

Treatments:
There are two main aims of treatment for ichthyosis:

•Keeping the skin moisturised and supple – the main aim is to prevent cracks from forming in the skin as these can allow micro-organisms to penetrate the barrier of the skin and so infection to establish. This is done using moisturising creams, and baths and lotions called emollients.

•Removing some of the layers of skin cells, either by mechanical means (rubbing gently with a pumice) or by treatments such as alpha hydroxy acids (AHAs), which help to break up the chemical ‘cement’ that glues skin cells together.
There may be other treatments aimed at the cause of ichthyosis if this can be identified – for example correcting nutritional deficiencies, stopping or changing medication or treating hormonal problems.
Life Style & Home Care:
Although self-help measures won’t cure ichthyosis, they may help improve the appearance and feel of damaged skin.  These measures can be considered to help:

*Take long soaking baths to soften the skin. Then use a rough-textured sponge, such as a loofa sponge, to remove the thickened scales.

*Choose mild soaps that have added oils and fats. Avoid deodorant and antibacterial soaps, which are especially harsh on dry skin.

*After showering or bathing, gently pat or blot your skin dry with a towel so that some moisture remains on the skin.

*Apply moisturizer or lubricating cream while your skin is still moist from bathing. Choose a moisturizer that contains urea or propylene glycol — chemicals that help keep your skin moist. Petroleum jelly is another good choice. Cover the treated areas with plastic wrap to keep the petroleum jelly from staining clothes and furniture.

*Apply an over-the-counter product that contains urea, lactic acid or a low concentration of salicylic acid twice daily. Mild acidic compounds help your skin shed its dead skin cells. Urea helps bind moisture to your skin.

*Use a portable home humidifier or one attached to your furnace to add moisture to the air inside your home.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

 

Resources:
http://www.mayoclinic.com/health/ichthyosis-vulgaris/DS00734
http://www.beltina.org/health-dictionary/ichthyosis-treatment-severe-diagnosis.html
http://en.wikipedia.org/wiki/Ichthyosis
http://www.bbc.co.uk/health/physical_health/conditions/ichthyosis.shtml
http://www.skinsite.com/info_ichthyosis.htm

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