Bullous epidermolysis is a rare hereditary genetic disease that causes the formation of painful bubbles on the skin and anywhere on the body, which can even appear in the mouth, eyes, palm and soles.
Symptoms of bullous epidermolysis may appear after friction or trauma caused by irritation of the laundry or when removing a band-aidfor example.
In the presence of symptoms indicative of bullous epidermolysis, it is recommended to consult the dermatologist to evaluate and indicate proper treatment, which includes drainage of bubbles and dressing in the skin bubbles, for example.
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Bullous epidermolysis symptoms
The main symptoms of bullous epidermolysis are:
- Bubbles on the skin, which break forming wounds;
- Bubbles inside the mouth and eyes;
- Cicarization of the skin with rough appearance;
- Small white spots on the skin;
- Deformities in the nails of the hands and feet;
- Decreased hair;
- Reduced sweat or excess sweat.
These symptoms vary according to the type and severity of bullous epidermolysis, but usually get worse over time.
According to the severity of bullous epidermolysis there may still be the formation of scars on the fingers of the hands and feet, leading to deformities.
Also read: Bubbles with transparent liquid on the skin: 14 causes (and how to treat)
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Does bullous epidermolysis hurt?
Yes, bullous epidermolysis hurts anywhere where it has bubbles, such as palms, mouth, eyes, elbows, knees and soles.
Pain in bullous epidermolysis is often more intense in people who have the most severe form of this disease.
Also read: Bubbles in the hands that scratch: 7 causes (and what to do)
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How the diagnosis is made
The diagnosis of bullous epidermolysis should be made by the dermatologist from the evaluation of the signs and symptoms presented, the performing of skin biopsy and the immune examination.
Biopsy along with the immune test are important to evaluate the skin in more detail and thus make the most accurate diagnosis of epidermolysis.
Make an appointment with the nearest dermatologist to make the diagnosis and start the most appropriate treatment:
Cause of Bullous Epidermolisis
Bullous epidermolysis is caused by transmitted genetic mutations from parents to child, and may be dominant, when one parent has the disease gene, or recessive, in which the father and mother carry the disease gene but there is no manifestation of signs or symptoms of the disease.
Children who have close relatives with the disease or bullous epidermolysis gene are more likely to be born with this type of condition.
Therefore, if parents know that they have the disease gene through genetic tests, it is indicated to do genetic counseling.
Is bullous epidermolysis contagious?
Bullous epidermolysis is not contagious, that is, it is but from one person to another through contact with skin lesions.
Main types
According to the skin layer where bubbles form, the different types of bullous epidermolysis are:
1. Simple Bullous Epidermolysis
In simple bullous epidermolysis, bubble formation happens in the upper layer of the skin, called the epidermis, being common in the hands and feet.
In this type it is possible to observe the rough and thick nails and the bubbles do not heal quickly.
2. Dystrophic bullous epidermolysis
In dystrophic bullous epidermolysis, bubbles arise due to defects in the production of type V | I collagen and occur in the most superficial layer of the skin, known as dermis.
3.
The junctional bullous epidermolysis is characterized by the formation of bubbles due to the detachment of the region between the most superficial and intermediate layer of the skin.
In this case, the disease occurs by mutations in the genes linked to the dermis and the epidermis, such as Laminin 332.
4. Kindler syndrome
Kindler syndrome is also a type of bullous epidermolysis, but is very rare and surrounds all layers of the skin, causing extreme fragility.
How treatment is done
Since there is no cure, treatment is done to relieve symptoms and reduce the chances of emerging new bubbles, including supporting measures such as wound dressings and pain control.
In some cases, hospitalization is required to be made sterile dressings, free of microorganisms, and to administer medications directly in the vein, such as antibiotics or corticosteroids, such as prednisone or hydrocortisone, to combat infections and decrease inflammation.
It can also be indicated to perform the drainage of the bubbles, which must be done by a health professional. Bubbles should be drilled with a specific needle and sterilized compresses. After drainage, it is important to apply a product, as spray Antibacterial, to prevent infections.
In addition, nutritional follow -up can also be indicated by the doctor for cases where it is not possible to feed properly.
When surgery is required
Surgery for bullous epidermolysis is usually indicated for the case where the scars left by the bubbles make body movement difficult or cause deformities that reduce the quality of life.
In some cases, surgery can be used to do skin grafts, especially in wounds that are taking a long time to heal.
How to Dress
Curative is part of the routine of those who have bullous epidermolysis and these dressings should be done carefully to promote healing, reduce friction and avoid bleeding in the skin.
For this, it is important to use non -adherent products on the skin, that is, they do not have glue that fixes very strongly.
To make wound dressing that have a lot of secretion it is important to use dressings made of polyurethane foam as they absorb these fluids and offer protection against microorganisms.
In cases where wounds are already dry, it is recommended to use hydrogel dressings as they help eliminate dead skin tissue and relieve pain, itching and discomfort on site.
Dressings should be fixed with tubular or elastic meshes, not being indicated to use adhesives on the skin. See more details on how to dress up.
What to do to avoid the emergence of bubbles
Some recommendations to avoid the emergence of the bubbles are:
- Wear cotton clothes, avoiding synthetic fabrics;
- Remove labels from clothes;
- Avoid clothing with sewing;
- Wear light, wide and comfortable shoes;
- Use a soft lock to dry the skin, pressing the gently;
- Apply Vaseline before removing dressings and not forcing your withdrawal;
- Cover the wounds with a non -adhesive dressing and loose gauze;
- Sleep with socks and gloves to avoid sleeping injuries;
- Take a bath with warm water, avoiding very hot water.
Also, in babies, diapers should not contain elastics and hygiene can be done with liquid paraffin or oil -based emollient.
Possible complications
Bullous epidermolysis can cause some complications such as infections, as bubble formation make the skin more susceptible to be contaminated by bacteria and fungi, for example.
In some more serious situations, these bacteria that enter the skin of the person with bullous epidermolysis can reach the bloodstream and spread to the rest of the body, causing the sepsis.
Also read: Sepsis: what it is, symptoms, types, causes and treatment
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People with bullous epidermolysis may also suffer from nutritional deficiencies, which arise due to lesions bleeding.
Some dental problems, such as caries, may appear, as the mucosa of the mouth is very fragile in people with this disease. In addition, some types of bullous epidermolysis may increase risk of skin cancer development.
Source: www.tuasaude.com