Fixed Implant-Supported Full-Arch Prosthesis in Epidermolysis Bullosa With Severe Symptoms.

Abstract

E pidermolysis bullosa (EB) is a group of rare hereditary mucocutaneous disorders that is characterized by the presence of a mechanical defect between the skin and the mucosa membranes with recurring appearances of blisters and vesicles. This disorder is classified in 4 principal groups—Simplex, Junctional, Dystrophic, and Kindler syndrome—and 32 subtypes. Recessive dystrophic with generalized impact presents most frequently in the oral mucosa. Systemic features of this disease include blisters on the entire body—particularly in areas of friction, such as the hands, the feet, the elbows and the knees. The blisters subsequently burst, leaving painful ulcers that heal via contraction of the soft tissue. In its most severe presentations, junctional and dystrophic EB, this contraction of the soft tissue often results in digital syndactyly, with the formation of stumps and stenosis of the superior third of the esophagus; this, in turn, causes dysphagia. Oral symptoms include the formation of recurring blisters and scars, limited oral aperture, ankyloglossia, obliteration of the buccal grooves, microstomia, severe periodontal disease and resorption of the alveolar bone, maxilla atrophy with mandibular prognathism, increased mandibular angle, and a predisposition to oral carcinoma. Routine dental care and regular brushing of the teeth can also cause blisters and lesions in the oral mucosa. In some cases, rampant caries associated with hypoplastic enamel and poor oral hygiene are observed. The treatment of EB patients with conventional removable dentures has resulted in development of blisters in the oral mucosa as a result of mechanical friction of the prosthesis over the tissues. As a result, restoration using dental implants and a fixed prosthetic is likely to be better tolerated by the patient, as it limits the possibility of lacerations and blisters in the soft tissue. The objective of this clinical case is to describe the restoration of the complete arch in an atrophic maxilla using endosseous implants and an implant-supported fixed prosthetic in a patient diagnosed with RDEB who is exhibiting severe general and oral symptoms. We will also evaluate improvements in the patient’s quality of life using a subjective scale of satisfaction.