Dermatology

Ichthyosis

​Overview:

  • Ichthyosis refers to a group of skin disorders characterized by persistently red, cracked, dry, thickened, 'fish scale' skin. 
  • There are at least 20 varieties of ichthyosis, including inherited and acquired forms in adulthood.
  • There is currently no cure for this condition; however, a daily skin care routine can make the symptoms milder and more under control.
  • You can alleviate the symptoms by: showering, maintaining a cool room temperature at home, and wearing clothes that do not irritate your skin.
What is ichthyosis?
It is a skin disorder characterized by persistently red, cracked, dry, thickened skin. 

Other names:
Fish scale 

Types: 
Ichthyosis vulgaris: It is the most common type of ichthyosis.1 in every 250 people have it.
The other types of ichthyosis are rare. They include:
X-Linked Ichthyosis: It occurs only in males, and includes general scaling, especially on the limbs and torso.
Congenital ichthyosis: Ichthyosis may arise if a child is born with a bright yellow membrane (collodion membrane), which is a sticky fluid that falls out during the first week of an infant’s life. Once the membrane falls off, one of the following types of ichthyosis can develop:
  • ​Non-bullous ichthyosiform erythroderma
  • Bullous ichthyosiform erythroderma 
  • Lamellar ichthyosis
In severe cases of bullous ichthyosiform erythroderma, the baby may suffer from drooping lower eyelids, loss of light hair, and tight skin on fingers.
Harlequin ichthyosis: It is extremely rare; but causes severe scales, and requires intensive care at birth.
Syndromes associated with ichthyosis: Netherton syndrome and Sjogren-Larson syndrome, for example.
Acquired ichthyosis: It tends to develop in adulthood and is not hereditary. Moreover, it is usually related to other conditions such as:
  • Underactive thyroid gland disorder
  • Kidney disease
  • Sarcoidosis: It is a rare condition that causes small patches of red, swollen tissue to appear on the body's organs.
  • Lymphoma (Hodgkin disease). It is a rare type of cancer.
  • HIV
Some medications can also trigger ichthyosis, including medications used in targeted cancer therapy (e.g. Vemurafenib and protein kinase inhibitors).

Cause:
Alterations and mutations in one or more specific genes lead to ichthyosis. This altered gene may be passed on from one or both parents to their baby or mutate on its own. The symptoms of ichthyosis appear at birth or during the first year of life. 
Defective genes affect the rate of skin regeneration, and lead to the buildup of rough, scaly skin. This is caused by one of the following conditions: Either the shedding of old skin cells is very slow, or the skin cells are reproducing at a much faster rate than they can shed. Some people may also develop ichthyosis as a result of another medical condition or a side effect of a medication.

Risk factors:
Anyone can develop ichthyosis. The disease usually spreads genetically. However, it can appear for the first time in a family due to one of the aforementioned reasons.

Symptoms:
Symptoms of ichthyosis range from mild to severe. The most common symptoms include:
  • Dryness of the skin
  • Itching, redness, and flaking of the skin
  • Scales on the skin that are white, gray, or brown
Depending on the type of ichthyosis you have, other symptoms that appear may include:
  • Blisters that can turn into wounds
  • Hair loss or breakage
  • Difficulty closing eyelids and dry eyes
  • Inability to sweat because the scales of the skin clog the sweat glands
  • Difficulty hearing
  • Thickening of the skin on the palms of the hands and the soles of the feet
  • Skin tightening
  • Difficulty bending some joints
  • Open wounds from scratching itchy skin
  • Symptoms are often worse in cold and dry conditions, and better in warm and humid conditions. This may cause them to be more noticeable in winter than in summer.
When to see a doctor?
  • If you suspect that a person or child has ichthyosis, make an appointment with your family doctor or dermatologist to get the condition diagnosed.
  • Seek medical attention if your symptoms worsen or do not improve with self-care measures. You may need stronger medication to control the condition.
Complications:
  • Fever: Resulting from the inability to sweat
  • Limited movement: Dry skin can make moving certain parts of your body very painful.
  • Skin infection: Cracked skin may lead to infection.
  • Hearing or vision impairment: This happens when there is skin buildup over the ears or eyes.
Diagnosis:
There is no specific examination for ichthyosis. Doctors usually diagnose the condition using:
  • Family and medical history
  • Physical examination of the skin, hair, and nails
  • Skin biopsy to examine tissues under a microscope
  • Genetic test to see if there is an altered gene
Treatment:
There is no cure for ichthyosis. The goal of the treatment plan is to manage the condition by moisturizing and exfoliating the skin daily. This can help prevent dryness, flaking, and skin cell buildup. The treatment plan may include:
  • Creams and ointments to moisturize the skin
  • Medications that help reduce scaling
  • Antibiotics or antiseptics to treat skin infections
Your dermatologist may prescribe or recommend proper moisturizing treatments, which may be in the form of a cream, ointment, lotion, or shower oil. He might also recommend exfoliating creams (e.g. salicylic acid) to help exfoliate and moisturize your skin.

Tips to control the disease:
Skin care:
  • Apply emollients to wet skin to lock in moisture, preferably a few minutes after showering.
  • Gently rub the wet skin with a pumice stone to remove some of the thick skin.
  • Comb washed hair to remove scales from your scalp.
  • Keep the temperature of the room cool, especially if you can't stand the heat and have decreased sweating or lots of itching.
  • Use a humidifier to keep moisture in the air and prevent the skin from drying out.
  • Wear loose-fitting clothes made of materials like cotton which may be less irritating to your skin.
  • Use laundry detergents designed for sensitive skin. Avoid the types that contain dyes or fragrances.
  • Find support from people close to you. Some people may find it helpful to talk to a therapist about coping with this disease.
Myths & Realities:
Ichthyosis is a contagious disease. 
Truth: Ichthyosis is not contagious.  

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Last Update : 26 November 2020 08:37 AM
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