From the Guidelines
Pyostomatitis vegetans is primarily caused by inflammatory bowel disease (IBD), particularly Crohn's disease and ulcerative colitis, as evidenced by the strong association between IBD and this rare oral condition 1. This rare oral condition is considered a specific marker for IBD, with approximately 75% of cases occurring in patients with underlying intestinal disorders. The exact pathophysiology involves an abnormal immune response where neutrophils accumulate in the epithelium, creating characteristic pustules and vegetative lesions in the mouth. Other potential causes include nutritional deficiencies, medications, and immunosuppressive conditions. Key factors that contribute to the development of IBD, and subsequently pyostomatitis vegetans, include:
- Genetic susceptibility
- Abnormal mucosal immune response against luminal bacteria
- Environmental factors, such as diet and stress Treatment focuses on addressing the underlying IBD through medications like corticosteroids, immunosuppressants such as azathioprine or methotrexate, and biologics like infliximab, as recommended in the management of IBD 1. Topical treatments including corticosteroid rinses, tacrolimus, or antiseptic mouthwashes may help manage oral symptoms. The condition typically improves when the underlying bowel disease is controlled, highlighting the importance of a multidisciplinary approach involving both gastroenterologists and oral medicine specialists for effective management. It is essential to note that the management of IBD has evolved, with a focus on achieving induction of remission, followed by maintenance therapy to prevent recurrent disease flares, and the use of biologics and small molecules like tofacitinib has improved outcomes for patients with IBD 1.
From the Research
Causes of Pyostomatitis Vegetans
The exact cause of pyostomatitis vegetans is unknown, but several factors have been suggested as possible etiological factors, including:
- Immunological factors 2
- Microbial factors 2
- Inflammatory bowel disease, such as ulcerative colitis or Crohn's disease, which is often associated with pyostomatitis vegetans 2, 3, 4
- Genetic predisposition, as suggested by a report of familial pyostomatitis vegetans 5
Association with Inflammatory Bowel Disease
Pyostomatitis vegetans is a highly specific marker for inflammatory bowel disease, and its correct recognition may lead to the diagnosis of ulcerative colitis or Crohn's disease 2, 4. The association between pyostomatitis vegetans and inflammatory bowel disease is well-established, and the management of pyostomatitis vegetans is usually based on the management of the underlying bowel disease 4.
Other Possible Factors
Other possible factors that may contribute to the development of pyostomatitis vegetans include: