All you need to know about Ichthyosis Vulgaris.
Know your ailment well, so you can manage it better!!
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What is Ichthyosis?
Ichthyosis is a category of skin disorders leading to swollen, itchy, scaly, rough and red skin. Signs can vary from mild to extreme. Ichthyosis may only affect the skin but certain types of the disease can also damage internal organs.
Most people inherit ichthyosis from a mutated (altered) gene from their parents. Some individuals, however, develop a form of acquired (nongenetic) ichthyosis from another medical condition or certain medications. Though ichthyosis is currently not healed, research is underway, and medications are available to help relieve the symptoms.
The outlook for people with ichthyosis differs according to the form and nature of the disease. Most people with ichthyosis require ongoing care to help make the disorder more manageable.
What are the types / forms of Ichthyosis:
Ichthyosis Vulgaris is the most common type. It is usually mild and appears in the first year of life with dry, flaky skin.
There are more than 20 features of ichthyosis, including those that occur as part of another syndrome or condition. Doctors may determine the type of ichthyosis by identifying the:
- Gene mutation.
- Inheritance pattern through analyzing family trees.
- Symptoms, including their severity and which organs they affect.
- Age when symptoms first appeared.
Some types of the disease, which are inherited and are not part of a syndrome, include the following:
- Harlequin ichthyosis is usually seen at birth and causes thick scaly plates of skin that cover the entire body. This form of the disorder can affect the shape of facial features and may limit joint movement.
- Epidermolytic ichthyosis is present at birth. Most infants are born with fragile skin and blisters covering their body. Over time, the blisters disappear, and scaling of the skin develops. This can have a ridged appearance over areas of the body that bend.
- Lamellar ichthyosis is present at birth. The infant is born with a tight clear covering the entire body, called a collodion membrane. Over several weeks, the membrane peels away, and large, dark, plate-like scales develop over most of the body.
- Congenital ichthyosiform erythroderma is present at birth. Infants also often present with a collodion membrane.
- X-linked ichthyosis usually develops in males and begins at about 3 to 6 months of life. Scaling is usually present on the neck, lower face, trunk, and legs, and symptoms can worsen over time.
- Erythrokeratodermia variabilis usually develops a few months after birth and progresses during childhood. The skin can develop rough, thick or reddened areas of skin, usually on the face, buttocks, or limbs. The affected areas can spread on the skin over time.
- Progressive symmetric erythrokeratodermia usually appears in childhood with dry, red, scaly skin primarily on the limbs, buttocks, face, ankles, and wrists.
What are the symptoms of Ichthyosis :
Symptoms occur in the inherited forms at birth or during the first few months of life.
The principal symptom is rough, scaly skin. The scales are built on only certain parts of the body, such as:
- Trunk
- Stomach
- Buttocks
- Legs
- Face and scalp
The scales may be white, dark brown or grey. Maybe they run through them with thick or thin cracks. In cold , dry weather the dryness and scaling get worse. Usually they benefit when the weather is warmer. Other indications of ichthyosis include:
- Skin redness
- Blisters
- Peeling
- Itchiness
- Pain
- Lines on the palms of the hands and soles of the feet
- Tight skin that makes it hard to move
Many people with ichthyosis also have eczema, a red, itchy rash.
What Causes Ichthyosis?
Ichthyosis Vulgaris is commonly caused by a genetic mutation that’s inherited from one or both parents. Children who inherit a defective gene from just one parent have a milder form of the disease. Those who inherit two defective genes have a more severe form of ichthyosis Vulgarisomplications:
What are the Complications of Ichthyosis?
Skin is the barrier to your body. It holds inside moisture, and it keeps out bacteria and other invaders that could make you sick. You lose some of that protective layer when ichthyosis makes pieces of your skin scale off. Scaling up can lead to complications like:
- Infections
- Dehydration
- Blocked sweat glands, which can lead to overheating
- Slow hair growth from scales on the scalp
- Burning more calories, because the skin has to work harder to turn over cells.
Ichthyosis can also be causing emotional problems. Scales can have an effect on how you look. Sometimes people with this condition feel depressed, and have low self-esteem. Also men with ichthyosis are at greater risk for:
- Testicular cancer
- Undescended or hidden testicles
- Low sperm count
Other complications include:
- Vision loss from damage to the cornea (the clear layer in the front of the eye)
- Brain and nervous system problems
Who Develops this disease?
Anybody gets ichthyosis. Usually the disease is passed down from your parents; however, due to a new gene mutation some people may be the first in a family to develop ichthyosis. Other people develop an acquired form of ichthyosis (nongenetic), which results from another medical condition or a side effect of a medication.
How to diagnose Ichthyosis?
Health care providers usually diagnose ichthyosis by:
- Ask about family history and medical history and any skin conditions.
- Completing a physical examination which includes closely examining the skin, hair , and nails.
- Doing a skin biopsy for microscopic examination of the tissue. Doctors often use a biopsy to help diagnose the infection, or to decide if the symptoms arise from another disease or skin disorder.
Additionally, your health care provider may be able to diagnose ichthyosis with a genetic test that will usually detect the mutated gene from a blood sample or a mouth swab. A genetic counsellor or specialist could help you understand the results of the tests.
What is the treatment for Ichthyosis?
Ichthyosis vulgaris has no proven cure so the treatment purpose is to control the disease.
Medications:
- Exfoliating creams and ointments: Prescription creams and ointments that contain alpha hydroxy acids, such as lactic acid and glycolic acid, help reduce the scaling and increase moisture in the skin.
- Oral medication: Your doctor can prescribe medications derived from vitamin A, called retinoids, to reduce skin cell development. Medication side effects may include irritation of the eyes and mouth, bone spurs and hair loss.
- Retinoids may be responsible for birth defects. Before beginning the drug, women seeking retinoid therapy should be confident that they are not pregnant-and use successful birth control when taking retinoids.
Lifestyle and Home Remedies:
Self-help measures may help improve the appearance and feel of damaged skin. Consider these suggestions:
- Take long soaking baths to soften the skin. Use mild soap. Rub dampened skin lightly with a rough-textured sponge (loofa) or a pumice stone to help remove the scales.
- After showering or bathing, gently pat or blot the skin dry with a towel so that some moisture remains on the skin.
- Apply moisturizer or lubricating cream while the skin is still moist from bathing. Choose a moisturizer with urea or propylene glycol — chemicals that help keep skin moist. Petroleum jelly is another good choice.
- Apply an over-the-counter product that contains urea, lactic acid or a low concentration of salicylic acid twice daily. Mild acidic compounds help skin shed its dead skin cells. Urea helps bind moisture to skin.
- Use a portable home humidifier or one attached to your furnace to add moisture to the air inside your home.
References:
https://www.webmd.com/skin-problems-and-treatments/what-is-ichthyosis#1
https://www.mayoclinic.org/diseases-conditions/ichthyosis-vulgaris/diagnosis-treatment/drc-20373759
https://www.niams.nih.gov/health-topics/ichthyosis
By,
Gopala Krishna Varshith,
Content Developer & Editor,
Clipo.
