Progressive Systemic Sclerosis and its Dental Implications: A Case Report

Scleroderma is a complex autoimmune rheumatic disease characterised by the widespread fibrosis of skin and visceral organs, dysregulation of immune system and vasculopathy. The aetiology of this disease remains unknown till now. It is known to exist in two forms namely localised and generalised/progressive. It is known to affect females more commonly than males, and also shows a wide range of oral and paraoral manifestations including xerostomia, periodontal disease, restricted mouth opening, burning mouth syndrome, dysphagia, gastroesophageal reflux. A 76-year-old female patient presented with the chief complaint of dry mouth, restricted mouth opening, burning sensation in mouth and reduced taste perception for the past seven years. On the basis of gastrointestinal disorder, Raynaud's phenomenon, fish mouth appearance and various other extraoral and intraoral features a diagnosis of Progressive Systemic Sclerosis (PSS) was made. The present case also emphasised upon the crucial role of a general dentist in diagnosing such rare systemic diseases. This disease is known to carry a significant burden in the form of pain and disability which potentially limits the proper functioning of major internal organs and results in various life limiting complications. Thus, early diagnosis with appropriate investigations followed by specialist and multidisciplinary approach to treatment is the key to management of the disease.