Zmiany patologiczne błony śluzowej jamy ustnej występujące w wybranych chorobach o podłożu genetycznym u pacjentów w wieku rozwojowym – przegląd piśmiennictwa

Abstract

A number of systemic disorders of genetic origin increase the patient's susceptibility to the occurring pathological changes in the oral mucous membranes. Most often these lesions occur in early childhood and may be one of the decisive factors leading to identification of a particular disease entity.The aim of this article is to present the type and characteristics of the lesions on the oral mucosa occurring in patients of developmental age with selected congenital disorders and make clinical recommendations useful for both dentists and pediatricians.For the purpose of this article, a large number of diseases were selected, which often manifest themselves in the oral mucosa, and the general practitioner can contribute to a more rapid child's diagnosis. The existence of the genetic disease may prove particularly the occurrence of chronic and recurrent lesions with severe intensity.After a review of the literature, lesions were classified to 3 groups: lesions caused by hereditary vascular abnormality (Sturge-Weber syndrome, Rendu-Osler-Weber syndrome, Maffucci syndrome), proliferative changes (Hereditary gingival fibromatosis, Juvenile hyaline fibromatosis) and other oral mucosa changes in selected diseases of genetic origin (Peutz-Jeghers syndrome, Down syndrome, Epidermolysis bullosa).Oral mucosa lesions in these patients can be both specific and nonspecific (infectious, pigmentary, hyperplastic). Clinical management is varied, and depends on the specific disease entity, the current state of the general patients health, location, and the nature and severity of lesions in the oral cavity. Pediatrician cooperation with a dental team allows an individual treatment plan. In all children diagnosed with systemic genetic condition, it is very important to take care about maintaining proper oral hygiene, regular dental visits to an appropriate specialist, and parental/guardian involvement in prophylactic and therapeutic treatment.