Ichthyosis and Dehydration of the Skin

The most important function of the human epidermis is to establish and maintain an effective barrier function that allows transepidermal water loss to a small degree but protects against dehydration. Additionally, the skin also provides a natural barrier against absorption of exogenous substances. This barrier function is reached by a complex differentiation process of epidermal cells which develop by division of active basal cells and turn into dead, flattened squames in the stratum corneum that are tightly connected to each other and further surrounded by lipid layers.

Defective epidermal differentiation and cornification are observed in various skin disorders, predominantly the ichthyoses, a group of skin diseases characterized by a generalized scaling of the skin and skin dryness.

Dry skin is caused either by

• a genetic defect which may have been spontaneous (due to a mutation of a gene) or passed on through family inheritance, whereby a protein called filaggrin which is normally found in large quantities in the outermost layers of the skin is reduced or absent leading to impaired formation of the skin barrier (the skin's natural shedding process is slowed or inhibited and, in some forms the production of skin cells is too rapid). This manifests in three skin disorders with dry, scaly skin: ichthyosis vulgaris, atopic dermatitis (eczema) and psoriasis. Major Gene Discovered That Causes Dry Skin And Leads To Eczema And Asthma
• a reduction in the concentrations of the skin’s water-holding sugars and proteins, and
• damage to the skin’s protective barrier.

Gene Linked Dry Skin?
Ichthyosis (ick-thee-o-ses) is the name of a rare genetic fillagrin skin disorder.  Since it's a genetic mutation, it isn't contagious--you can't "catch" it. Ichthyosis causes the skin to build up and scale, causing it to be extremely dry, among other problems.

Most types of ichthyosis are present at birth, and are life-long.  Currently, there is no cure, only treatments. As a result, the skin dries out too easily and in addition, the outer layers of the skin are poorly formed and constantly flake off. 

As well as keeping water in, the skin barrier normally keeps foreign substances out of the skin. In people with filaggrin mutations, foreign substances can easily enter the skin and be seen by the immune system. This explains the development of inflamed skin (eczema). In some people, priming of the immune system through the "leaky" skin appears to lead to asthma when foreign substances later enter the lungs.

Worldwide, about 60 million people are estimated to carry these particular gene defects and more than 1 million are predicted to have the severe form of the disease as a consequence of these mutations alone.

Dryness is exacerbated by wind, extremes of temperature and air-conditioning, all of which cause the skin to flake, chap and feel tight.  Skin looks dull, especially on the cheeks and around the eyes. There may be tiny expression lines on these spots and at the comers of the mouth.  Chapping and cracking are signs of extremely dry, dehydrated skin. 

What does a dehydrated skin feel like? It is usually dry to the touch, itchy, with flaky patches and rough spots, and blotchy patches when you look in the mirror. Your skin tone is dull and pale. The skin loses its elasticity wherein when you apply a bit of pressure on the skin, it does not revert back to its original state. It usually feels "tight" and uncomfortable after washing.

Dry skin also has a low level of sebum production and provides little lubrication to the skin.

Genetically speaking, about 10% of the population is affected by the inherited form of dry skin caused by a genetic mutation known as ‘ichthyosis’ (fish-scale skin) and may be disfiguring enough to cause psychological distress.

The mutated gene causes an abnormality in the normal lifecycle of skin. Whilst in most people, the growth, dying and shedding of skin happens unnoticed, people with ichthyosis reproduce new skin cells at a rate faster than they can shed it, or reproduce at a normal rate but the rate of shedding is too slow. Either way there is a build up of dry scaly skin.

The dry areas may result in dermatitis, i.e. the skin becomes red and itchy. This may result in a crazy-paving appearance on the lower legs (‘eczema craquelé’), or round patches scattered over the trunk and limbs (a dry form of nummular dermatitis).

Sometimes the dry skin is just itchy, without much of a rash (sometimes known as ‘winter itch’, ‘7th age itch’, or ‘senile pruritus’).

Dry skin is often just a temporary problem — one you experience only in winter, for example — but it may be a lifelong concern. And although skin is often driest on your arms, lower legs and the sides of your abdomen, this pattern can vary considerably from person to person. What's more, signs and symptoms of dry skin depend on your age, your health status, your locale, the amount of time you spend outdoors, and the cause of the problem.

There are various kinds of ichthyosis.
Ichthyosis is characterised by persistently dry, thickened, rough, "fish scale" skin. There are at least 20 varieties of ichthyosis, including inherited and acquired forms. (http://dermnetnz.org/scaly/ichthyosis.html)Ichthyosis vulgaris, accounts for 95% of all ichthyosis cases. Inheritance is autosomal dominant i.e. it is passed from a parent to about half their children. Signs and symptoms of ichthyosis vulgaris usually become apparent within the first year of life. The other main forms of inherited ichthyosis include lamellar ichthyosis, epidermolytic hyperkeratosis, and X-linked ichthyosis.

What are some of the problems associated with ichthyosis?
These may vary depending on the type of ichthyosis. Some people with ichthyosis may experience some or all of the following:

• Overheating: Many idividuals with ichthyosis, do not sweat normally. The thickness of their skin and the scale does not allow their sweat to reach the surface of their skin and cool them effectively.
• Limitations in movement: The tightness of the skin may make it painful to move some parts of the body.
• Secondary infection: Splitting and cracking of the skin can lead to skin infections and/or systemic infections.
• Impaired eyesight or hearing: Skin build up over the eyes or ears may occur.

Acquired ichthyosis is not inherited and occurs for the first time in adulthood. It is usually associated with some general systemic disease, such as underactive thyroid states, sarcoidosis, lymphoma, generalised cancer or HIV infection. It may be provoked by certain medications (such as kava, nicotinic acid and hydroxyurea).

Dermatologists often call dry skin arising in later life ‘xerosis’, ‘asteatosis’, ichthyosis vulgaris or ‘acquired ichthyosis’.

If you have dry skin due to normal aging, you're likely to experience one or more of the following:

• A feeling of skin tightness, especially after showering, bathing or swimming
• Skin that appears shrunken or dehydrated
• Skin that feels and looks rough rather than smooth
• Itching (pruritus) that sometimes may be intense
• Slight to severe flaking, scaling or peeling
• Fine lines or cracks
• Redness
• Deep fissures that may bleed

Ordinary dry skin (xerosis) or dehydrated skin lacking water usually isn't serious, but it can be uncomfortable and unsightly, turning plump cells into shriveled ones and creating fine lines and wrinkles.

Chronic alcohol abuse also causes dry skin (ichthyosiform erythroderma), as a consequence of alcohol-induced diuresis. Erythroderma refers to the skin that is diffusely red and inflamed with varying degrees and types of scaling. There are many causes of erythroderma, but the most common are exacerbations of an underlying skin disease, underlying malignancies, dermatoses, infections, and drug reactions. Erythroderma is a rare, potentially serious skin condition. Protein loss in the form of desquamation and exudation is significant. Biopsy analysis is important to rule out cutaneous T-cell lymphoma. People with such a condition apart from scaling and erythema also suffer from pruritus and should be carefully evaluated for underlying diseases. Erythroderma can represent a serious medical threat, and may require hospitalization.

If you feel your skin is dry and tight, and your skin tone is ashen and dull you may either have a dry skin type that is dehydrated or have an oil skin type which is dehydrated.

Unfortunately, the moisturizers and treatments claiming to alleviate dry skin do nothing to cure it. To be able to correct dehydrated skin, a drastic change in lifestyle and environment is required.

Fortunately, most dry skin results from environmental factors that can be wholly or partially controlled. These include exposure to hot or cold weather with low humidity levels, long-term use of air conditioning or central heating, and excessive bathing.

Some of these factors can easily be remedied and others will take more time and effort.

When you leave your skin dehydrated for a long period of time, keratinization occurs. Take for example the feet. The skin on the bottom of your feet is subjected to much heat, friction and dirt. The dead skin cells that build up on the surface of your skin forms a coat covering it, making it nearly impossible for moisturizers to reach your skin. Your skin will further get dehydrated. Then keratinization occurs with breakouts since your skin is badly in need of water.

Many moisturizers and emollients sold by major skin care companies delay the healing of irritated and damaged skin and make the situation worse by inhibiting natural skin repair. New computerized instruments have demonstrated that many popular moisturizers increase skin damage in methods similar to skin irritants. Nor are skin barrier creams an answer, such as those containing petrolatum and lanolin.

What we need is to protect the skin surface and to heal the skin from within, by putting the skin in a situation in which normal skin repair can occur.

What really works is to protect the skin surface and to heal the skin from within, by providing the skin the nutrients an environment in which normal skin repair can occur. Please continue here for more about how to relieve dry skin

Last Modified: August 14, 2009

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