Definition of Epidermolysis Bullosa
Epidermolysis bullosa is a rare group of diseases that causes the skin to become brittle and prone to blisters. The appearance of blisters on the skin can be triggered by minor injuries, sun exposure, friction with clothing, or scratching.
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| Epidermolysis Bullosa, Causes, Diagnosis, Treatment, Prevention, Complications |
Epidermolysis bullosa can cause painful blisters on the skin of the hands, elbows, knees and feet. However, these blisters can also appear on the skin inside the body, such as the mouth, throat or eyes.
Symptoms and Causes of Epidermolysis Bullosa
Apart from blisters on the skin, epidermolysis bullosa is also accompanied by other symptoms, such as thickened skin on the palms or soles of the feet, thickening of the fingernails or toenails, and the appearance of small white bumps.
Epidermolysis bullosa is a rare skin disorder inherited from parents with the condition. This condition occurs due to damage to the gene that produces collagen.
Treatment and Prevention of Epidermolysis Bullosa
Treatment of epidermolysis bullosa is carried out by administering drugs, physiotherapy, and surgery. To help the recovery process, patients can also do self-care at home, such as changing bandages regularly and applying petroleum jelly cream.
Epidermolysis bullosa cannot be prevented. However, there are several ways you can do to prevent more serious health problems, namely wearing soft and loose clothing, and eating foods that are not too hot.
Causes of Epidermolysis Bullosa
Epidermolysis bullosa is caused by damage to the genes that make collagen. This affects the function of collagen in maintaining the strength and structure of the skin. As a result, the epidermis and dermis skin layers do not bind together so that the skin is easily brittle and chafed.
A person can suffer from epidermolysis bullosa if one or both parents suffer from the disease.
Based on where the blisters appear, epidermolysis bullosa is divided into several types, namely:
1. Epidermolysis Bullosa Simplex
Epidermolysis bullosa simplex is the most common type of epidermolysis bullosa. In this type, blisters may appear on the skin of the newborn's hands and feet. Blisters that have improved later will usually thicken and harden.
2. Dystrophic Epidermolysis Bullosa
Dystrophic epidermolysis bullosa can form blisters on the skin of the hands, feet, knees and elbows. However, these blisters can become severe and widespread, even causing the skin to peel.
Dystrophic epidermolysis bullosa occurs when the collagen that provides strength to the dermis layer of the skin is lost or does not function. As a result, the layers of skin cannot blend properly.
3. Epidermolysis bullosa junctional
This type can cause blisters on the skin of a newborn's hands, elbows, knees, and feet. Blisters will usually decrease when the baby is 2-3 months old. However, blisters that heal can leave scars and cause the toes or fingers to join together.
Junctional epidermolysis bullosa is the most severe type and can increase the risk of a baby dying in the first year of life.
4. Kindler Syndrome
Kindler syndrome is a very rare type of epidermolysis bullosa. Babies with Kindler syndrome may experience blisters on the skin of the backs of their hands and feet. In some conditions, Kindler syndrome can also cause sufferers to become sensitive to sunlight.
Kindler syndrome can increase the risk of developing squamous cell carcinoma. Squamous cell carcinoma is a skin cancer that forms in squamous cells, which are cells that form the middle and top layers of the skin.
5. Epidermolysis Bulosa Acquisita
Unlike other types, epidermolysis bullosa acquisita is classified as an autoimmune disease, which is a condition when the body's immune system actually attacks collagen, causing blisters on the skin.
While other types of epidermolysis bullosa appear when a newborn is born, epidermolysis bullosa acquisita generally occurs when a person is 30–40 years old. Blisters of this type can appear on the hands, knees, elbows, and ankles.
Symptoms of Epidermolysis Bullosa
Common signs and symptoms of epidermolysis bullosa include:
- Blisters on the skin of the hands, feet, elbows, or knees
- Blisters in the mouth and throat
- Thickened skin on the palms or soles of the feet
- Cavities are caused by poor enamel formation
- Attached fingers or toes
- Thickened fingernails and toenails
- Small white bumps on the skin (milia)
- Difficulty swallowing (dysphagia)
- Developmental disorders
When to See a Doctor
Immediately consult a doctor if you or your child experience the above symptoms or blisters appear on the skin for no apparent reason. Medical help must be done immediately if you experience the following complaints:
- Difficult to swallow
- It's hard to breathe
- Signs of infection, such as warm, swollen skin, pus, foul-smelling sores, and fever
Diagnosis of Epidermolysis Bullosa
To diagnose epidermolysis bullosa, the doctor will perform a physical examination based on the patient's complaints. After that, the doctor will carry out further examinations, in the form of:
- Skin biopsy
- A skin biopsy is done by taking a sample of the patient's skin to be examined under a microscope.
- Genetic test
- Genetic tests are carried out by taking a patient's blood sample. The goal is to identify the gene that causes the type of epidermolysis bullosa in the patient
Treatment of Epidermolysis Bullosa
Epidermolysis bullosa treatment methods include administering drugs, physical therapy (physiotherapy), and surgery. The following is an explanation:
Drugs
Administration of drugs aims to relieve pain and itching, as well as overcome sepsis. Doctors can also prescribe oral antibiotics if the patient's wound shows signs of widespread infection, such as fever, weakness, and swollen lymph nodes.
Physiotherapy
Scar tissue and blisters on the skin can restrict limb movement. Therefore, the doctor will suggest physiotherapy to improve the patient's mobility.
Operation
If drugs and physiotherapy are not effective in treating epidermolysis bullosa, the patient will be advised to undergo surgery. The surgical procedure aims to:
- Dilates the esophagus
- Blisters in the esophagus (gullet) can cause the esophagus to narrow, making it difficult for sufferers to eat. Esophageal dilation surgery can be performed to make it easier for food to pass through the esophagus to the stomach.
- Restore movement
- This surgical method is performed to treat fingers or toes that are fused due to blisters and scar tissue that keep appearing. Thus, the patient can return to activities.
- Provide nutrition
- The doctor will insert a gastronomic tube into the stomach through the outer wall of the stomach. The goal is for food to go directly into the stomach. That way, patients who have difficulty eating can still get nutritional intake.
- Replacing blisters
- Skin graft surgery may be performed if epidermolysis bullosa affects the function of the affected body part.
In addition to some of the medical treatments above, patients can make several efforts to speed up the recovery process, namely:
- Wear soft and loose clothing
- Avoiding direct sun exposure, as it can make blisters worse
- Take pain relievers prescribed by your doctor regularly
- Wash hands before touching blisters and changing bandages
- Applying a moisturizing cream, such as petroleum jelly, to the blister and covering it with a bandage
- Do not peel the broken blisters and let them dry to protect the layers of skin underneath
- Do not scratch or rub the skin hard
- See a doctor if there are signs of infection, such as a red rash or pus in the blisters
If blisters appear in the mouth or esophagus of babies and children, parents can take several ways to fulfill their nutritional intake. Efforts that can be made include:
- In babies who are still breastfeeding, use a special pacifier or pipette.
- For children, serve nutritious foods that are soft and easy to swallow, such as vegetable soup or porridge.
- Give food that is warm, and avoid giving food that is too hot.
- If necessary, consult a doctor to provide food supplements to children.
Epidermolysis bullosa complications
Epidermolysis bullosa that is not treated immediately can cause various health problems, such as:
- Constipation
- Bacterial infection
- Infections that cause low blood pressure and damage to multiple organs (sepsis)
- Dental problems, such as the appearance of tartar or cavities
- Fingers or toes fused
- Formation of scar tissue in joints that interfere with movement
- Malnutrition
- Anemia
- Blind
- Skin cancer
- Death
- Prevention of Epidermolysis Bullosa
Epidermolysis bullosa cannot be prevented. However, because this disease is hereditary, genetic testing is recommended for prospective parents who have a family history of epidermolysis bullosa.
In addition, there are several efforts that can be made to prevent skin blisters and infection in patients with epidermolysis bullosa, namely:
- Keep the skin moist by applying petroleum jelly
- Wear special shoes with a soft texture
- Make sure you or your child's nails are kept short or wear gloves to prevent infection from scratching the wound
- Provide a soft mat, especially on the buttocks and back of the neck, if you are going to carry a child with epidermolysis bullosa
- Does not lift the child from under the arm
- Remove the elastic on the diaper and line the inside of the diaper with a non-stick bandage
- Covering hard surfaces with soft materials, for example placing a thick towel in a child's bath
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