Most cases of ichthyosis are hereditary and begin in childhood. In rare instances, adults can acquire the condition as a side effect of certain medications or other medical conditions.
Currently, there is no known cure for the condition, but the consistent and regular use of moisturizers and exfoliants are often enough to resolve symptoms.
What is ichthyosis vulgaris?
Ichthyosis vulgaris is the most common form of ichthyosis, a group of skin conditions.
Image credit: Skoch3, 2012
Ichthyosis vulgaris is a type of ichthyosis, a group of related skin conditions that interfere with the skin's ability to shed dead skin cells, causing extremely dry, thick skin.
Extremely dry, scaly skin is known as xerosis.
There are more than 20 different types of ichthyosis, but ichthyosis vulgaris is considered the most common form. It accounts for nearly 95 percent of all ichthyosis cases.
Ichthyosis is a relatively common condition, impacting approximately 1 in every 250 people. It usually develops in early childhood, typically between the ages of 2 and 5.
Ichthyosis vulgaris is often called fish scale disease because the scales that characterize the condition look like fish scales.
A doctor diagnoses the condition by looking at the skin changes and determining if anyone else in the family has a similar problem. The doctor will likely also ask about other medical issues and perform a skin biopsy or obtain a cheek cell sample. Genetic testing is also often used to confirm the condition.
What is harlequin ichthyosis and is it serious?
Harlequin ichthyosis is a rare form of ichthyosis that is present at birth. A newborn with the condition will have thick plates of skin that crack and split apart. Eating and breathing may be difficult, and infection may occur.
In the past, it was difficult to survive more than a few days with this condition. Now, however, treatment is available. If applied immediately, it can be effective. Some children with harlequin ichthyosis are already living into their teens and 20s.
Most cases of ichthyosis vulgaris are caused by a mutation in the gene responsible for encoding filaggrin. This is a protein that helps create the skin's natural barrier.
Without an effective barrier, the skin struggles to retain moisture and a consistent pH.
Chronically dehydrated skin cells begin to thicken and harden as they age. They then move to the surface of the skin, where they become fixed scales.
A vast majority of ichthyosis cases are inherited. Individuals with one copy of the abnormal gene tend to have milder cases than those with two copies.
The condition can also be caused by:
- the use of certain medications
- systemic conditions
- conditions that affect the entire body
Common causes of acquired ichthyosis include:
- impaired thyroid function
- Hodgkin lymphoma
- organ failure, especially liver and kidney failure
- many medications targeted cancer therapy drugs
- medicines containing nicotinic acid
- medications containing kava
Ichthyosis vulgaris most commonly affects the torso, head, and limbs.
Ichthyosis vulgaris causes extremely dehydrated skin resulting in thick and scaly skin.
The most commonly impacted regions of the body include:
- extensor region of the limbs, especially the elbows and shins
- face, typically the forehead and cheeks
Typically, symptoms are not present at birth but develop at around 2 months of age. Initially, symptoms are often mistaken for dry skin.
Ichthyosis vulgaris may present as skin dryness with accompanying fine, white, or skin-colored scales. Often, the skin will also flake.
The scaling associated with ichthyosis vulgaris can also cause the skin to crack in areas that are severely or persistently affected. Cracking most often occurs on the soles and palms.
Scaling can also lead to general discomfort and pain and make the skin more vulnerable to irritation and further drying. While rare, in some people with ichthyosis vulgaris, scaling interferes with the sweat glands causing either excessive sweating (hyperhidrosis) or an inability to sweat.
The severity of symptoms varies from person to person and symptoms can intensify during puberty. Anything that causes the skin to dry out further, or impairs its ability to retain moisture may also worsen symptoms.
Individuals with both conditions have an increased risk of:
Most people with both ichthyosis vulgaris and eczema tend to present with severe symptoms at a young age that persist into adulthood.
Ichthyosis vulgaris is also commonly associated with keratosis pilaris, a condition where hair follicles become clogged with skin scales.
Ichthyosis vulgaris is also associated with hyperlinearity, or increased skin lines, often on the soles and palms.
Unfortunately, there is no known cure for ichthyosis vulgaris. Treatment options revolve around lessening symptoms by removing scaling and reducing skin dryness.
Regular, consistent exfoliation aims to slowly dull scales. Once scaling subsides, the skin is better able to take in and retain moisture.
Treatment of ichthyosis vulgaris involves removing scaling. This can include gentle exfoliation of the dry skin.
Commonly recommended ways to reduce scaling include:
- soaking the affected area in salt water or bathe in salt water
- soaking the affected area in lukewarm water and then gently rubbing in a circular motion using a pumice stone
- using moisturizers that contain exfoliating chemicals, such as glycolic acid, alpha hydroxy acid, lactic acid, salicylic acid, or urea
- carefully brushing washed hair to remove scaling on the scalp
- for severe cases, using oral vitamin A-based medications like acitretin or isotretinoin to slow down skin cell production
Alongside exfoliation, using moisturizers and lotions consistently and regularly:
- helps increases skin hydration
- reduces the risk of cracking and splitting
- may prevent further scaling
It is a good idea to use lotions or body washes that have a high-fat content, such as lanolin creams, as the first line of treatment.
A variety of environmental factors and lifestyle habits dehydrate the skin, intensifying the dryness and scaling associated with ichthyosis vulgaris.
Some easy ways to reduce the likelihood of worsening symptoms include:
- avoiding environments with cold or dry air
- taking short showers or baths
- cutting down on the frequency of bathing
- avoiding harsh or scented soaps, detergents, body washes, shampoos, and conditioners
- blotting, rather than rubbing the skin after exposure to water
- applying moisturizers immediately (within 3 minutes) after showers or baths
- after moisturizing, cover the skin with sealants, such as plastic wrap for a few hours to physically trap moisture and increase saturation
- avoiding heavily air-conditioned environments
- avoiding places controlled by centralized heating
- using a humidifier
- protecting the skin from wind or cold
- avoiding environments with high air pollution that can interfere with the skin's natural pH
- avoiding the use hard tap water that interferes with the skin's pH
- using sunscreen, ideally those with added moisturizers
- avoiding tanning beds or artificial tanning
- avoiding pools and hot tubs that contain skin drying chemicals and irritants, such as chlorine
- treating other skin drying conditions, such as eczema