Prevention of oral diseases in children with epidermolysis bullosa: a case report and literature review

Abstract

Today, more than 300 million people worldwide suffer from one or more rare diseases. Epidermolysis bullosa (EB) is a heterogeneous group of hereditary diseases characterized by a genetic predisposition to a hypersensitive body reaction to minor skin damage, characterized by the formation of blisters and erosions on the skin and mucous membranes, followed by scarring. It is one of the most severe hereditary dermatoses. In EB, pathological conditions also occur in the oral cavity. Thus, patients with EB require a special “atraumatic” approach to medical procedures, hygienic care, professional oral hygiene, etc. Purpose - to systematize clinical recommendations for oral care for patients with BE on the basis of a clinical case. Clinical case. We present a clinical case of a patient with a dystrophic EB and a diagnosis of acute chronic granulating periodontitis. Examination of the oral cavity revealed microstomia, multiple caries, erosions and ulcers on the oral mucosa, signs of chronic catarrhal gingivitis, as well as dentoalveolar anomalies. After achieving a satisfactory degree of mouth opening using a set of exercises for the maxillofacial muscles, treatment of the acute chronic granulating periodontitis of the tooth 23 was performed. As a result of the clinical observation and analysis of the literature, we systematized practical recommendations aimed at facilitating the treatment of oral diseases in patients with EB. Conclusions. Treatment of oral diseases in patients with EB is primarily aimed at preventing pain and preventing secondary infection of affected areas. Treatment of dental caries contributes to the correct position of the tongue, improved swallowing and phonetics, as well as to improvement in nutritional status and aesthetic appearance. Oral hygiene reduces the risk of injury to the oral and pharyngeal mucosa. In the treatment of patients with EB, it is important to use a multidisciplinary approach with the involvement of specialists of different specialties. Implementation of simple but effective recommendations made by pediatric dentists will increase the effectiveness of prevention of oral diseases in these children. The study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent of parents and child was obtained for the study. No conflict of interests was declared by the authors.