Hussna Khan

Abstract

Introduction: This case describes an 84-year-old patient who suffers from pemphigus vulgaris. It aims to share the long-term management of a patient who presented with a failing dentition and the additional complexities of prosthodontic rehabilitation posed by pemphigus vulgaris and long-term corticosteroid use. It also aims to review issues associated with long term bisphosphonate use and the risk of medication related osteonecrosis of the jaw (MRONJ).

Case report and discussion: The patient presented with a mucosa supported partial denture which was exacerbating the pemphigus vulgaris. The patient was referred by the Oral Medicine department due to her failing maxillary dentition and for consideration of an implant retained prosthesis. In 2007 the remaining maxillary teeth were extracted and 9 months later, 4 implants were placed in the maxilla. Six months following implant placement, a milled titanium bar incorporating CEKA attachments was fixed to the 4 implants and cast cobalt chrome frameworks were utilised in the maxilla and mandible. In the years following, the patient was regularly reviewed. Maintenance involved repairs of CEKA attachment screws, as well as replacement of the bar and eventual replacement of the dentures. Pemphigus vulgaris is characterised by flaccid intraepithelial bullae which rupture to create areas of painful ulcerations and erosions. Oral pemphigus vulgaris often involves areas exposed to mechanical irritation which is why the buccal mucosa is considered the most common site. Autoantibodies (IgG) bind to desmosomes especially desmoglein 3 (Dsg3). This antigen-antibody complex deactivates the adhesion function of Dsg3 and causes separation of suprabasilar cells leading to acantholysis and blister formation. The resulting tissue fragility poses challenges for prosthodontic management. If left untreated, pemphigus vulgaris has the potential to be fatal which is why early diagnosis and treatment can have a positive impact on the prognostic outcome. Prednisolone is a corticosteroid which the patient has been taking for almost 30 years. Most recent guidance suggests that patients taking 5mg or more of prednisolone for one month or longer may be at risk of adrenal suppression secondary to systemic glucocorticoid use. Treatment with short term glucocorticoids causes a rapid and significant decrease in type I procollagen N-terminal propeptide and osteolactin which are markers for bone formation.  It has been suggested that glucocorticoids have a role in modulating bone cell subpopulation. Increased concentrations of glucocorticoids can decrease the rate of bone formation as well as the number and activity of osteoblasts and osteocytes, alongside increasing osteocyte lifespan. Longer term corticosteroid therapy is also a risk factor for osteonecrosis of the jaw.

Conclusion: Rehabilitation of patients with a failing dentition is a challenge. When combined with oral manifestations of pemphigus vulgaris and a history of long-term bisphosphonate use, the challenge is even greater and requires a multidisciplinary approach to achieve a successful outcome. Guidance for the dental management of patients taking oral bisphosphonates doesn’t contraindicate dental implants but special consideration should be given especially for those who have been taking oral bisphosphonates for more than 3 years and with steroids such as prednisolone.