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Ichthyosis Vulgaris - Types, Causes, Diagnosis, and Treatment

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Ichthyosis is a collection of skin diseases that makes the skin extremely dry, thick, and scaly. The commonest type is ichthyosis vulgaris.

Medically reviewed by

Dr. Dhepe Snehal Madhav

Published At November 8, 2022
Reviewed AtMarch 20, 2023

Introduction:

Ichthyosis is a collection of skin diseases where the skin becomes extremely dry, thick, and scaly. The commonest and the mildest type is ichthyosis vulgaris. Other rare types include harlequin ichthyosis, lamellar ichthyosis, and x-linked ichthyosis. Ichthyosis vulgaris often begins in childhood which can be inherited or acquired. The skin cells accumulate producing thick flakes that look like fish scales. Whether ichthyosis vulgaris develops in children or adults, it is commonly mistaken for extremely dry skin. Many people do not diagnose ichthyosis because after applying moisturizer the skin becomes free of scale.

What Are the Types of Ichthyosis Vulgaris?

  1. Inherited Ichthyosis Vulgaris – it is caused by genetic inheritance from one or both parents. The disease appears during childhood, between three months and five years of age.

  2. Acquired Ichthyosis Vulgaris – it is a very rare type of ichthyosis usually affecting only adults. It occurs when triggered by a disease or a medicine.

Who Gets Ichthyosis Vulgaris?

Ichthyosis vulgaris can occur in any of the following conditions:

  • Genetic inheritance from one or both parents.

  • Mutation of genes causing ichthyosis during fetal life.

  • Any disease or medicine that triggers ichthyosis.

  • Both male and female of all races get ichthyosis vulgaris.

What Causes Ichthyosis Vulgaris?

Inherited type of ichthyosis vulgaris is caused by genetic changes. The gene is either inherited from one or both parents or mutation of genes occurs during the development of the fetus in the womb. Therefore there is decreased filaggrin in the skin that is required to produce a healthy outermost layer of skin. Without adequate filaggrin, the body cannot shed the superficial skin cells and accumulate older skin cells resulting in scale formation and skin thickening.

Acquired ichthyosis vulgaris has an underlying disease or a medication that triggers it. Diseases that trigger ichthyosis are long-term kidney failure, sarcoidosis, cancer especially lymphoma, and infections such as leprosy and HIV. Acquired ichthyosis vulgaris can indicate before the person has signs of the causing disease. If so, complete physical rest and identifying the cause is necessary. Rarely, medicines or vitamins can trigger acquired ichthyosis vulgaris. It includes Cimetidine, Clofazimine, Nicotinic acid (vitamin B).

What Are the Clinical Features of Ichthyosis Vulgaris?

Ichthyosis vulgaris often begins in childhood between three months and five years of age.

The following changes are seen:

The skin is white, gray, or brown, dry, curled edges, rough and scaly. Scales usually occur in the scalp, backs of the arms, legs, back, or belly. Thickened skin on the palms and soles that look dirty. If the lesion is severe, deep cracks on the palms and soles may be present that can cause infection, and inability to sweat normally. The skin becomes itchy. Rough bumps develop on the arms, buttocks, and thighs. This condition is known as keratosis pilaris. Ichthyosis vulgaris in adults is caused by a disease or medicine. The signs and symptoms remain the same as in children. Ichthyosis vulgaris may be associated with kidney disease, cancer, or human immunodeficiency virus (HIV). In children, the scale becomes more visible until puberty and then lessens. The lesion may reappear during the teenage or adult. The lesion worsens in the winter as the air is cold and dry. During hot and humid climates, ichthyosis vulgaris is hardly noticeable or completely fades off.

How to Diagnose Ichthyosis Vulgaris?

Ichthyosis vulgaris is diagnosed by the following ways:

  • Complete personal and family history.

  • Complete medical history.

  • A thorough physical examination.

  • A skin biopsy is done when the lesion resembles some other skin lesion.

How to Treat Ichthyosis Vulgaris?

There is no remedy for inherited ichthyosis vulgaris. Treatment is aimed at reducing the dry skin and scales.

  • Taking baths often as soaking helps hydrate and soften the scale in the skin. place petroleum jelly or any similar product on open sores before getting into the water. This reduces the burning and stinging sensation caused by the water. Adding table salt or sea salt to the water may also reduce the itch.

  • Gently rub the scale with buff puff, abrasive sponge, or pumice stone after soaking in water as it softens the scale in the skin.

  • Apply moisturizer to damp skin after bathing as it seals water from a bath or shower into the skin. Moisturizer containing active ingredients like urea, lactic acid, or alpha hydroxy acid helps reduce scale.

  • Applying petroleum jelly to the deep cracks can help get rid of it.

  • Antibiotics are prescribed in case of infection.

  • Retinoids, a derivative of vitamin A, may be prescribed in difficult cases to slow the production of skin cells. If ichthyosis is mild, treatment may be required only during the winter. In humid climates, ichthyosis improves naturally and reappears during winter.

What Are the Self Care Tips for Ichthyosis Vulgaris?

To help patients with this long-term skin condition and to get the better treatment results follow these selfcare tips:

Follow the treatment plan. Continuing proper baths, applying moisturizer, and reducing scale can maintain ichthyosis vulgaris under control. If the scales start to develop, remove it gently.

What Are the Complications of Ichthyosis Vulgaris?

Children with ichthyosis vulgaris have a higher risk of developing the following conditions:

  • Atopic dermatitis.

  • Hives.

  • Asthma.

  • Hay fever.

  • Secondary infection may occur due to skin splitting and cracking.

  • Overheating may occur as the skin thickens and interferes with sweating. This inhibits cooling. Sometimes, excess sweating can also occur.

  • Patients may also have psychological disturbances because of the appearance of skin.

What Is the Prognosis of Ichthyosis Vulgaris?

For inherited types of ichthyosis vulgaris, the prognosis is excellent. Most people live to their normal lifespan. Ichthyosis vulgaris becomes less serious on aging. However, most people require continued treatment of their skin for a lifetime. For acquired ichthyosis vulgaris, avoiding the triggering factor can treat the disease.

Conclusion:

Ichthyosis vulgaris is a group of skin diseases where the skin becomes dry secondary to excessive water loss. Although sometimes acquired, most ichthyoses are inherited and due to genetic mutations. Management depends on severity of the disease and treatment includes topical medications and lifestyle modifications with or without oral retinoids. Genetic counseling also plays an important role.

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Dr. Dhepe Snehal Madhav
Dr. Dhepe Snehal Madhav

Venereology

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