Butterfly skin, how to learn to live with this rare disease
When someone listens on their television or reads in a newspaper about certain weird illness It seems that they are dealing with a subject that will never touch them at home. However, although the frequency of these conditions is not as frequent as others more common, there are and when they make an appearance in a family, parents do not know how to cope with this situation.
It's the example of butterfly skin, weird illness which is characterized as a set of conditions, whose genetic origin is multifactorial. Among the consequences can be noted nutritional alterations and delayed growth and weight. Experts say that over time the patients of this rare disease usually improve, so an early diagnosis is recommended to start the relevant treatment as soon as possible.
Diagnosis of butterfly skin
The first thing parents should know about butterfly skin is that there is not a single typology of this rare disease. From the Ministry of Health The following classes of this condition, also called Epidermolysis Bullosa, are listed below:
- epidermolytic or simple epidermolysis bullosa. In these cases, the blisters are located intraepidermally, just in the cells of the basal layer.
- EpidermolysisBullosa of the junction or junctions. The ampulla of these patients is located at the level of the basement membrane.
- dermolytic or dystrophic epidermolysis bullosa. These patients usually show the blister being below the basement membrane at the level of the anchoring fibrils.
For the diagnosis of all of them the first step of medical exam It should be performed in all those dermatoses present in newborns that are characterized by the presence of blisters and erosions. The doctors will interview with the parents to know their family history, medicines taken by the mother during the pregnancy or the newborn, existence of infections in the pregnancy or of the neonate, etc.
There will also be a Clinical examination to locate the lesions, the mucous membrane involvement or the existence of infections. As a complementary test, a skin biopsy will always be performed, obtaining sufficient skin for conventional immunohistochemical, immunofluorescence and electron microscopy studies.
In order to detect their early presence, in recent times specialists are betting on their prenatal diagnosis in order to detect this condition before the child is born. In particular, in cases where there is a history of Epidermis Bullosa, it is recommended to perform some of these tests:
- Fetal skin biopsy
- Preimplantation diagnosis
- Prenatal ultrasound
Evolution and treatment
As has been said, the prognosis of patients with Epidermolysis bullosa is good and over time their standard of living improves considerably thanks to the treatments. Therefore it is essential to detect it in time to begin to alleviate the symptoms that characterize this disease. Among the most used techniques:
- Dressings on the blisters to relieve pain and promote healing.
- Surgical treatments to remove blisters in cases where their presence is more bothersome.
- Use of astringent lotions and topical antibiotics such as fusidic acid, mupirocin or gentamicin.
- Monitoring the weight and size of the smallest in order to control the side effects in the smallest related to their weight.
- Treatments with analgesics to avoid pain caused by the presence of blisters in the dermis of children.
- Replacement of the elements that are lost in cases such as anemia.
Damián Montero
