Congenital epidermolysis bullosa. Clinical features and own observations

Inherited epidermolysis bullosa is a group of genetic skin disorders characterized by skin erosions, ulceration, skin and mucosal blistering requiring topical treatment. This review demonstrates major clinical manifestations of epidermolysis bullosa and its mechanisms of development. According to these features the main principles of topical treatment and drug therapy were developed, including physical protection from trauma, moisturizing, improvement of wound healing, prevention and management of infection, itch and pain management. Drug therapy is outlined with dosage forms, drug routes of administration, age restrictions indicated in the instruction for medical use for the medications that could be used in epidermolysis bullosa patients. The authors provide indications for clinical use of antiseptics, disinfectants, antibiotics, antimicrobial agents, emollient cream and drugs reducing itch and pain.

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Topical treatment of inherited epidermolysis bullosa

Kubanov

Чикин

Карамова

et al. 2021

Vestnik dermatologii i venerologii

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Clinical observation of a newborn infant with congenital epidermolysis bullosa. The role of the nurse

Chernenkov,

Panina,

Plochoskay

2023

Medsestra

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Currently, there is a great need to expand knowledge regarding epidermolysis bullosa in newborns. This is due to the difficulty in differential diagnosis. Awareness of this disease will help to avoid errors in diagnosis and allow to prescribe the correct treatment to the patient. The aim of the work was to present a clinical case of congenital epidermolysis bullosa in a newborn baby born in 2022 in the Saratov region. The analysis of medical records, the course of the neonatal period of the patient diagnosed with congenital bullous epidermolysis was performed. Results. Characteristic features of clinic and diagnostics of the disease were singled out and described in this work on the example of the concrete clinical situation. Comparison of infectious and noninfectious skin diseases makes the main contents of the work. Particular attention has been paid to differential diagnostics because of its difficulty. Conclusion. Bullous exanthema is found in many inflammatory skin diseases; therefore, it is necessary to differentiate congenital bullous epidermolysis from neonatal vesicular disease and pigment incontinence syndrome. Awareness of the disease will allow to avoid mistakes in prescribing treatment to the patient, since the tactics of skin treatment for VTE and infectious skin diseases are different (use of alcohol-based aniline dyes is contraindicated). To improve the skin barrier and well-being of butterfly babies, proper care is necessary from birth. Protecting fragile skin from damage, treating and dressing wounds is probably the only thing that can help such children today. For external treatment, special dressings and ointments are used, in order to reduce pain and prevent infection. Today, more and more butterfly children have the opportunity to receive qualified assistance, necessary medications and dressings, thanks to the activities of the charity foundation «B.E.L.A. Butterfly Children».

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Congenital epidermolysis bullosa. Clinical features and own observations

Cited by 2 publications

References 8 publications

Topical treatment of inherited epidermolysis bullosa

Topical treatment of inherited epidermolysis bullosa

Clinical observation of a newborn infant with congenital epidermolysis bullosa. The role of the nurse

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