Ichthyosis Vulgaris

  • Medical Author:
    Gary W. Cole, MD, FAAD

    Dr. Cole is board certified in dermatology. He obtained his BA degree in bacteriology, his MA degree in microbiology, and his MD at the University of California, Los Angeles. He trained in dermatology at the University of Oregon, where he completed his residency.

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    William C. Shiel Jr., MD, FACP, FACR

    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

Ichthyosis vulgaris facts

  • Ichthyosis is a skin disease that superficially appears similar to fish skin with scales.
  • Ichthyosis is a heritable disease and cannot be transmitted from one person to another.
  • Ichthyosis produces very dry itchy skin because of a defective protein that is important in maintaining skin hydration.
  • Ichthyosis is relatively rare.
  • Keeping the skin well lubricated is an important part of the treatment for ichthyosis.

What is ichthyosis vulgaris?

Ichthyosis vulgaris is a rare inherited skin condition that is characterized by extremely dry skin (xerosis) involving most of the skin surface. Ichthyosis vulgaris produces large scales in the skin that can resemble fish scales, hence the term ichthyosis. Vulgaris simply means the common form. Other types of ichthyosis are even rarer. Patients with this condition have a defective skin barrier, which permits the evaporation of water at an elevated rate.

What are causes and risk factors for ichthyosis vulgaris?

An inherited genetic defect, a mutation, affecting a giant protein called filaggrin causes ichthyosis vulgaris. One inherits the defective gene from one's parents and may be produced by one or more mutations in the profillagrin gene produced early in fetal development. It is expressed in an autosomal dominant fashion, which means that all that is necessary to have the disease is a single defective copy of the gene that codes for filaggrin. Since this protein plays a major role in the maintenance of the correct level of hydration in the skin, affected patients have difficulty retaining water in the superficial layers of the skin. A significant number of patients with atopic dermatitis, asthma, and hay fever share mutations in this gene, as well.

Causes of Dry Skin

There is no single cause of dry skin. Dry skin causes can be classified as external and internal. External factors include cold temperatures and low humidity, especially during the winter when central heaters are used. Internal factors include overall health, age, genetics, family history, and a personal history of other medical conditions like atopic dermatitis.

External factors that cause dry skin include

  • over-washing with harsh soaps,
  • overuse of sanitizers and cleaning agents (alcohol),
  • cold temperature,
  • low humidity.

Is ichthyosis vulgaris contagious?

Since this is an inherited condition, it is not contagious and cannot spread from person to person.

What are ichthyosis vulgaris symptoms and signs?

The most significant finding, which is ultimately diagnostic, is the presence of very dry skin over most of the skin surface. The abnormal appearance of the skin is diagnostic of ichthyosis vulgaris. This dryness appears as rather large dry scales that seem to spare moist areas like the armpits and groin, as well as other flexural areas. The characteristic appearance is usually absent at birth but becomes obvious as the child ages.

How do health care professionals diagnose ichthyosis vulgaris?

Since ichthyosis vulgaris is relatively rare (one in 250-1,000 children), only physicians specializing in the skin (dermatologists) or in genetic defects are equipped best to diagnose and treat this condition. Doctors often suspect a diagnosis purely on the basis of the skin's appearance. Evidence of other blood relatives with ichthyosis would support the diagnosis. Occasionally, it may be necessary to examine affected skin with a biopsy using light microscopy and even electron microscopy. Other types of ichthyosis can be confused with ichthyosis vulgaris.

What is the treatment for ichthyosis vulgaris?

Since the condition is rare, there are few well-controlled treatment trials. There is no way of replacing the defective filaggrin molecule. Living in a humid environment is very helpful in controlling the disease. Patients usually do well in places like Southeast Asia as opposed to Northern Europe. Using emollients on the skin that tend to suppress the evaporation of water can be very helpful. Effective emollients usually come in jars and are thick and do not flow. Topical medications that contain 10% lactic acid, 10% urea, and/or 10% glycerol (AmLactin, Lac-Hydrin, Lactinol, Lactinol-E, LactiCare, Ureacin-10, Aqua Care) can be very helpful, as well.

Are there any home remedies for ichthyosis vulgaris?

As most of the topical medications recommended for the control of this condition are available without a prescription, people can treat their mild disease without physician supervision. It is prudent to see a physician specialist to obtain an accurate diagnosis and follow the medical advice.

Is there a special diet for those with ichthyosis vulgaris?

There are no dietary manipulations likely to affect this condition

What are complications of ichthyosis vulgaris?

The major complications that occur are due to the damage that is produced by the excessive dryness characteristic of this condition. Dry skin is exceptionally susceptible to irritation. Irritated skin is red and itchy. Scratching skin predisposes to skin infections. In addition, there seems to be a predilection for the development of eczema (atopic dermatitis) and other allergic conditions, including asthma and hay fever. Currently, medical professionals do not understand the reason for this relationship.

What is the prognosis of ichthyosis vulgaris?

Ichthyosis vulgaris may be either mild or severe depending on the precise genetic defect (more than one mutation affecting filaggrin production), as well as ambient environmental factors (average relative humidity and temperature). Mild disease has an excellent prognosis, whereas severe disease may be difficult to control.

Is it possible to prevent ichthyosis vulgaris?

Once a conception occurs, it is not possible to prevent the development of ichthyosis at this time.

Are there support groups for people with ichthyosis vulgaris?

The following are ichthyosis support groups on the Internet:

The Foundation for Ichthyosis & Related Skin Types (FIRST)

Ichthyosis Support Group

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Medically Reviewed on 10/16/2018
References
REFERENCES:

McLean, W.H.I. "Filaggrin Failure – From Ichthyosis Vulgaris to Atopic Eczema and Beyond." British Journal of Dermatology 175 (Suppl. S2) 2016: 4-7.

Thyssen, J.P., E. Godoy-Gijon, and P.M. Elias. "Ichthyosis Vulgaris: The Filaggrin Mutation Disease." British Journal of Dermatology 168.6 (2013): 1155-1166.
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