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chthyosis, or “fishskin disease,” is a relatively uncommon, hereditary abnormality of the skin that afflicts more than one million Americans. Derived from the Greek word ichthys (fish), the term ichthyosis refers to the fish-scale appearance of the skin. The “Alligator Man,” “Lizard Woman” and “Porcupine Man” of old time circus sideshows were victims of ichthyosis.
Rather than a single disease entity, ichthyosis represents a family of related diseases. There are four distinct types of these coarse, rough, and scaly skin disorders all of which are characterized by an increase in the thickness of the upper layers of the skin. It is caused by a genetic defect that can be either inherited or spontaneous. It is not due to germs or viruses and is, therefore, not contagious.
Brought about by abnormalities in either protein or fat metabolism, these diseases are a result of either an excessive production of skin cells, or as a consequence of increased “stickiness” of the uppermost layer of the skin – the horny layer – which occasions an impairment in the normal shedding process. The result is dry, thickened skin that shrinks and cracks.
(The upper layer of the skin – the epidermis – is constantly regenerating itself: all its cells turn themselves over about every 28 days. Dead cells slough off as new ones push up in a perpetual and lifelong process of cell division. It takes about two weeks for the newborn cells to make their way to the outermost layer of the epidermis, where they remain, as dead and dying cells, for another two weeks until they are cast off. See the chapter on Dandruff, page 000.)
The two most prevalent types of ichthyosis are ichthyosis vulgaris and X-linked ichthyosis. In both types, the normal shedding process is retarded.
ICHTHYOSIS VULGARIS
he most common – or “vulgar” – type of ichthyosis, known appropriately as ichthyosis vulgaris, develops after the first three months of life and affects about one in every 300 people. It is inherited, may affect several members of a family, and is characterized by fine, white, branny scaling which appears most prominently over the trunk and outside surfaces of the arms and legs. The inside of the elbow and knee areas – the flexures – are not involved, but the palms and soles are often thickened. There is usually no itching or burning or other subjective symptoms; and while the chief complaints of this fishlike, scaly disorder are often only cosmetic in nature, there are other considerations that must be kept in mind.
Children with this condition suffer physical and psychological problems. The skin, which cannot hold moisture, can become tight and stiff; severely limiting the child’s ability to play like other children. Even simple tasks can, on occasion, be painful.
Ichthyosis vulgaris improves with age and with warm, moist surroundings, and is often associated with some form of allergy – eczema, hay fever, or asthma – and with dry skin of the palms and heels that is intensified in cold, dry weather.
X-LINKED ICHTHYOSIS
he second most common type of inherited scaly skin is called X-linked ichthyosis. This more severe variety affects about one in 4,000 males and is transmitted to sons by unaffected mothers. As in ichthyosis vulgaris, this variant results from retarded shedding of the uppermost layers of the skin.
Characterized by early onset – from birth until about one year – and generalized involvement, this condition displays large, coarse, adherent, “stuck-on” scales over the neck, trunk, buttocks, and outer portions of the extremities. When the neck is affected, it invariably has an unwashed appearance. X-linked ichthyosis becomes increasingly severe with the passage of time.
While there is no cure for ichthyosis, various therapies can greatly improve the appearance of the skin and relieve the excessive dryness and scaling. The goal of therapy is to keep the scales from building up and to retain moisture in the upper layers of the skin.
A prescription cream – Lac-Hydrin Cream 12% – has offered genuine relief in many sufferers for the past few years. Some of the over-the-counter alpha-hydroxy acids have also provided modest improvements. Use a mild cleanser, such as Cetaphil Cleanser, when cleaning the skin.
If the person lives in a warm, humid environment, little treatment is required for either type of ichthyosis just described. Keeping the relative humidity levels above 60 percent is vital.
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For further information about ichthyosis, contact: F.I.R.S.T. (Foundation for Ichthyosis & Related Skin Types) 650 N. Cannon Avenue – Suite 17 Lansdale, PA 19446 800-545-3286; 215-631-1411 E-mail: focus@scalyskin.org www.scalyskin.org In addition, there is a National Registry for Ichthyosis |
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| TREATING ICHTHYOSIS |
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The topical application of soothing, lotions, creams, and ointments immediately following comforting oil baths is all that is necessary in the milder cases. Deodorant and other harsh soaps are poorly tolerated.
Soaps
Cetaphil
Basis Soap
Oilatum Soap
Bath Oils
Aveeno Bath
Directions for use on the labels.
Emollient Creams and Lotions
Amlactin Cream or Lotion
Amlactin XL
Directions for use on the labels.
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