Ichthyosis - Causes, Symptoms, and Treatment

Introduction

The term ichthyosis means fish scale. The skin in this condition resembles the scales of a fish. Hence, it is also called fish scale or fish skin disease. Ichthyosis comprises about 20 skin conditions that result in dry skin and the formation of scales. The human skin has numerous uses, of which its role in forming a protective barrier is the most important. New skin cells are recruited in the skin's deep layers, and dead cells are eliminated from the superficial layers. In ichthyosis, either the dead cells are exfoliated at a slow pace from the superficial layer resulting in the accumulation of the top dry layer, or the formation of new cells in the deep layers is very slow such that excess water may be lost from the skin.

What Are the Types of Ichthyosis?

The following are the types of ichthyosis:

1. Ichthyosis Vulgaris: About one in every 250 people may be affected by ichthyosis vulgaris. Brown, gray, or white scales can appear in early childhood.

2. X-Linked Recessive Ichthyosis: About 1 in 6000 people are affected by this type, but only the males are affected. It can increase the risk of developing testicular cancer. Women can act as carriers and might face labor issues.

3. Congenital Ichthyosis: A bright yellow collodion membrane (a sticky fluid that falls out) can develop during the first week after birth. Following the exfoliation of the membrane, one of the following can develop:

• Bullous Ichthyosiform Erythroderma: Those babies with a severe type of bullous ichthyosiform erythroderma can suffer from tight skin on fingers, drooping lower eyelids, and light hair loss.

• Harlequin Ichthyosis: It is a very rare condition, and there can be severe scale formation such that the babies born with this condition might need intensive care at birth.

4. Acquired Ichthyosis: It is not genetically acquired but develops later in life, usually in adulthood. It is linked with other conditions like:

• Underactive thyroid gland disorder.

• Kidney disease.

• Sarcoidosis (a rare condition that results in the formation of small patches of red, swollen tissue on the organs).

• Lymphoma or Hodgkin's disease (a rare type of cancer).

• HIV (human immunodeficiency virus).

5. Non-Bullous Ichthyosiform Erythroderma: In this condition, the babies are born with a tight, clear sheath that envelops their skin, called a collodion membrane.

6. Lamellar Ichthyosis: In this type, the ichthyosis is due to the abnormal production of the outer dead layer (cornification) of the skin. Certain medications like those used in targeted cancer therapy, such as protein kinase inhibitors,Vemurafenib, and nicotinic acid (used to treat high cholesterol), can also trigger ichthyosis.

What Are the Causes of Ichthyosis?

The causes are:

Inherited ichthyosis can be acquired genetically from one’s parents. Genetic mutations can result in inherited ichthyosis. Genetic mutations have an impact on the proteins that safeguard the skin and keep it moist. They also have an impact on the quick shedding and regeneration of new cells. The symptoms usually manifest in early childhood. A child can have a severe form of the condition if both parents carry the specific gene and the child acquires the gene from both parents. Acquired ichthyosis manifests in adulthood, and the reason for this type is unknown. But, individuals with this condition can have accompanying conditions like:

1. Kidney disease.

2. Underactive thyroid gland.

3. Cancer like Hodgkin’s lymphoma (cancer of the lymphatic system).

4. HIV (human immunodeficiency virus) infection.

5. Sarcoidosis (development of tiny groups of inflammatory cells in various parts of the body).

In some cases, both parents may be carriers of the defective gene and might not express the symptoms themselves but can transmit this gene to their offspring. Very rarely, the genetic mutation can occur on its own (spontaneously) in the affected generation.

What Are the Signs and Symptoms of Ichthyosis?

The large body areas like the legs, arms, ears, torso (trunk), face, buttocks, or entire body may be affected. Common signs and symptoms are as follows:

1. Dry skin.

2. Itching.

3. Redness of the skin.

4. The skin can get cracked.

5. Appearance of white, gray, or brown scales on the skin that may look like the following:

• Small and flaky.

• Large, dark, plate-like scales.

• Hard, armor-like scales.

Other symptoms based on the type of ichthyosis are as follows:

• Blisters that have the potential to break, resulting in wound formation.

• Fragile hair or loss of hair.

• Difficulty hearing.

• Dry eyes and struggle to close the eyelids.

• Unable to sweat since the sweat glands can get clogged with skin scales.

• Thickening of the skin on the palms of the hands and soles of the feet.

• Skin tightening.

• Difficulty bending certain joints.

• Open wounds can develop as a result of skin scratching.

Scaling and skin dryness can aggravate in cold, dry weather. The symptoms can be better during the summer. Many individuals with ichthyosis can also have eczema (a skin condition characterized by a red, dry, and itchy rash on the skin).

How to Diagnose Ichthyosis?

At times, the condition can be mild and resembles normal-dry skin. One can consult a dermatologist (skin doctor) to get rid of the dryness and scaling. The physician may do a physical examination to check the appearance of the skin, nails, and hair. The physician might also inquire about the family and medical history. A biopsy may be done to examine a skin sample under a microscope. The saliva may be tested to determine any genetic changes responsible for causing certain types of ichthyosis.

How Can Ichthyosis Be Treated?

The condition cannot be gotten rid of permanently, but the symptoms can be managed as follows:

• The skin can be moisturized daily by using creams, lotions, or ointments.

• Skincare products that contain ceramides or cholesterol help keep the skin moist.

• Creams that contain any one of the ingredients, like alpha hydroxy acids, lanolin, propylene glycol, or urea, can be used.

• Applying the cream or lotion immediately after a bath can help retain moisture since the skin is damp right after a bath.

• Shampoos that contain salicylic acid or tar can be used on the scalp to relieve itching and minimize the formation of scales. Brushing the hair after washing to remove the scales from the scalp is advisable.

• One can also take baths in salt water, and the skin can be rubbed with a pumice stone.

• A humidifier can be used to maintain moisture in the air and to prevent the skin from getting dried up.

• One can wear loose-fitting cotton clothes that might cause less irritation to the skin.

• Laundry detergents that contain dyes or fragrances should be avoided. Instead, detergents that are suitable for sensitive skin should be preferred.

• Dead skin can be eliminated by using products that contain glycolic acid, salicylic acid, or lactic acid.

• In case of severe scaling and dryness of the skin, one might be prescribed an oral retinoid drug like Isotretinoin (Absorica, Sotret, or Claravis) or Acitretin (Soriatane). If necessary, antibiotics may be prescribed to treat or prevent skin infections. However, retinoids can result in side effects like dry mouth, upset stomach, and weak bones.

If the ichthyosis results from another health condition or medication, treating the underlying condition or changing the medication may help relieve the symptoms.

It is necessary to remember that one must not smoke or go near flames after using an emollient (moisturizer) as it contains a flammable substance called paraffin. Also, one must avoid using soap as it can dry the skin further. Instead, one can use an emollient on the affected skin.

What Are the Complications Associated With Ichthyosis?

The formation of scales can result in complications like:

• Infections.

• Dehydration.

• Overheating due to clogged sweat glands.

• More calories are burnt since the skin works excessively to turn over cells.

• Retarded hair growth from scales on the scalp.

• Men diagnosed with ichthyosis are susceptible to developing testicular cancer (cancer in the testicles), hidden or undescended testicles, and might have low sperm count.

• Damage to the clear layer in the front of the eye, called cornea, can result in vision loss.

• Problems in the brain and nervous system.

Conclusion

Ichthyosis is a skin condition that results in skin dryness and the formation of scales. It can be inherited or acquired during adulthood. Some may be severely affected by the condition, which can affect the entire skin and can result in the formation of blisters. The condition cannot be permanently eradicated but can be symptomatically managed. Ichthyosis does not spread from one person to another. A few individuals may be emotionally affected since the scales can affect one’s appearance and lower their confidence and self-esteem. They might even feel depressed. It is advisable to consult a dermatologist if one notices the above-mentioned symptoms.