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Image: Dr. Gutiérrez Ontalvilla (left) with Volunteers Asociación Debra (Fina Martínez Vázquez and her husband www.debra.es) with our nurse Maite Mielgo in charge of the cures of children with epidermolysis bullosa. Missing from the photograph is my colleague Dr. Eva López Blanco, also a plastic surgeon at the Hospital La Fe in Valencia.
What is epidermolysis bullosa or “butterfly skin”?
Epidermolysis bullosa or bullous epidermolysis is one of the so-called rare diseases that causes the formation of recurrent wounds and blisters on the skin at the slightest touch or rubbing, an inherited genetic disease that usually manifests itself at birth or already in the first months of life. Epidermolysis bullosa is the result of a genetic error in the proteins that attach the epidermis (the most superficial layer of the skin) to the dermis (the deepest layer).
The disease is hereditary, from parents to children. In some cases only one parent is involved in the transmission and in others it is both parents who pass the defective gene to their children. There are far fewer cases in which the disease is present in the child without either parent having passed it on.
There are three main forms of the disease:
- Epidermolysis bullosa simplex: the blisters are located in the epidermis and heal without tissue loss. It is the least severe, and those affected may improve with time.
- Junctional or junctional epidermolysis bullosa: blisters occur between the inner and outer layers of the skin. There are different degrees, from a lethal variety in the neonatal stage to one that improves with time.
- Epidermolysis bullosa (dystrophic): blisters occur in the deepest layer of the skin (dermis). When the wounds heal, they cause retraction of the joints, which makes movement difficult, as the wounds end up sticking the skin, for example, between the fingers. It is also characterized by blistering of the mucous membranes of the mouth, pharynx, stomach, intestine, respiratory tract, urinary tract, inside the eyelids and cornea.
The skin of those affected with epidermolysis bullosa is characterized by being fragile, weak, extremely sensitive and extremely vulnerable, so delicate that at the slightest touch the skin peels off, causing wounds and blisters that have the appearance of a large burn; for this reason, the disease is also known as butterfly skin.
The treatment of epidermolysis bullosa is aimed at minimizing the complications of wounds and blisters and improving the quality of life of those affected. For this purpose, bandages and topical treatments are applied to the affected areas. In addition, close monitoring for bacterial infections secondary to the blisters must be followed.
On the other hand, it is necessary to give nutritional support to those affected by epidermolysis bullosa, providing calcium, iron, proteins and nutrients that are deficient as a result of the disease.
Plastic, reconstructive and pediatric surgery are also sometimes necessary, especially if there is esophageal stricture (narrowing of the esophagus) and to correct syndactyly and hands that are completely enclosed.
In addition, epidermolysis bullosa patients are more likely to suffer from squamous cell carcinoma, which has a high mortality rate among younger people, and does not respond well to chemotherapy, radiotherapy or surgery.
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