Epidermolysis bullosa is a painful and inherited disease. And it is not spreading or contagious disease, but it is a chronic one. There is no specific or curative treatment.
Epidermolysis bullosa (EB), also known as crystal skin or butterfly skin, is a heterogeneous group of genetic alterations that present with skin fragility and the mucosa. It predisposes patients to the development of blisters and erosions after minimal friction.
The management of the disease consists of avoiding as much as possible the traumas that trigger injuries and preventing infection, and facilitating wound healing with the systematic use of bandages. Do you want to know more about this pathology? We will detail it below.
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Table of Contents
What are the Causes of Epidermolysis Bullosa?
As detailed in the journal Experimental Dermatology, epidermolysis bullosa encompasses rare, clinical, and heterogeneous genodermatoses. There are an estimated 500,000 cases worldwide.
The skin made up of the epidermis (most superficial layer) and the dermis (innermost layer). Therefore, the region where both layers fuse is called the basement membrane. There lies the central problem of the pathology, in that union of tissues.
The different types are defined based on the layer where the blisters generated and the protein that altered in it. The main ones are the following:
- Simplex (EBS): alteration of keratins 5 and 14.
- Dystrophic (EBD): the gene that codes for collagen VII is affected.
- Juntural (EBJ): laminin 5 alteration
Symptoms of Epidermolysis Bullosa
In addition to the typical blisters and erosions secondary to the skin’s mechanical fragility, the disease can lead to nail dystrophy or anonychia, which is the total lack of nails. The clinical presentation of epidermolysis bullosa changes according to the type in which its classification framed. However, many signs are common to all varieties.
Alopecia
In the presence of anemia, reversible telogen effluvium can occur. This is hair loss, also known as alopecia. In some patients, alopecia has a typical pattern.
The degree of capillary involvement varies considerably among patients with type XVII collagen deficiency. There may be gradual alopecia in areas of friction trauma and blisters, called scarring alopecia.
Wound Healing
Multiple factors, including the following, compromise the healing process:
- Foreign bodies that lodge in the skin.
- Bacteria that colonize.
- Deficiency of nutritional elements in dermal tissue.
- Tissue hypoxia, which is the lack of oxygen supply in the tissues.
Optimizing healing occurs by controlling these factors. Hence, in the lack of a remedy for this pathology, promoting skin wounds’ physiological recovery is essential. The American Journal of Pathology indicates in a 2017 review that repetitive blistering leads to prolonged wound healing.
Infections
Prevention of infection is the preferred strategy for managing patients with epidermolysis bullosa. Also, extensive ulcerated skin areas often show loss of the stratum corneum barrier and allow the penetration of microbes. So the accumulation of moisture on the surface increases bacterial growth.
Along with low nutritional status, there is a decreased resistance to infection. Therefore, Staphylococcus aureus and Streptococcus pyogenes are the most frequent etiological agents in the finding.
With large bare or scabbed areas, strict care must taken. So this regimen includes topical antibiotics and the maintenance of the areas covered with a self-adhesive dressing, as recommended by the International Journal of Dermatology.
Skin Tumors
The squamous cell carcinoma occurs, in most cases, multiple primary sites of chronic injuries. So the peak incidence of these tumors increases dramatically in the second and third decades of life.
Skin biopsies taken in studies conducted and reported in the International Journal of Molecular Sciences revealed the crucial role of chronic tissue damage in creating a permissive tumor microenvironment. Thus, this is the formation of a suitable stimulus for the neoplastic cells.
Extracutaneous Affectations
Due to the high caloric intake that patients have and the complications acquired in the context of permanent skin damage, some of the types of epidermolysis bullosa with severe blisters characterized by secondary damage to other organs and systems.
According to a publication of the Journal of the American Academy of Dermatology, they may accompanied by some of the following manifestations:
- Lack of weight growth in weight and height.
- Anemia.
- Osteoporosis.
- Cardiomyopathies.
To Take into Account of Epidermolysis Bullosa
Family is a significant influence, affection, and love being essential to improve patients’ quality of life with epidermolysis bullosa. This is regardless of the economic situation, difficulties, time, or emotional factors.
The coexistence of symptoms such as anxiety, depression, and behavioral disorders should considered, as they can compromise treatment strategies and aggravate symptoms. So there is no remedy for the condition, but the progress made in approaches that limit complications and increase patient comfort.
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