Dental Management for Recessive Dystrophic Epidermolysis Bullosa Child-Patient - การรักษาทางทันตกรรมในผู้ป่วยเด็กที่เป็นรีเซสซิฟดิสโทรฟิคอิพิเดอร์โมไลซิ บูลโลซา: รายงานผู้ป่วย

Abstract

                      Epidermolysis bullosa is a group of rare hereditary skin disorders characterized by the formation of bullae on the skin and mucous membranes.  Blisters arise spontaneously and easily at points of trauma.  Epidermolysis bullosa is classified into three main groups based on anatomical location and distribution of the lesions.  Dystrophic epidermolysis bullosa, one of three subtypes, is characterized by blister formation occurring beneath the entire demo-epidermal junction ( sublamina densa) and divided into two types, dominant and recessive, according to mode of autosomal inheritance.  Recessive dystrophic epidermolysis bullosa has two subtypes, localized and generalized.  In generalized form, bullae becomes blister and heals with severe atrophic scarring and contracture.The character and extent of oral involvement in generalized recessive dystrophic epidermolysis bullosa are more severe than other types.  Subsequent scar formation causes alteration of soft tissue texture, obliteration of oral vestibule, ankyloglossia and microstomia.  The teeth are prone to dental caries.  This is a case report of a child-patient with recessive dystrophic epidermolysis bullosa who had dental problems.  Dental management such as teeth extractions and restorations were performed under local anesthesia as well as preventive measures were rendered to improve his oral health.  Complications, precautions, and some problems in this case were discussed.

Key words:      child-patient, dental management, recessive dystrophic epidermolysis bullosa