A review published in the Journal of Pediatric Gastroenterology and Nutrition provides guidelines for the diagnosis and management of oral extraintestinal manifestations (EIMs) of pediatric inflammatory bowel disease (IBD). Authored by clinicians with experience in gastroenterology and oral and craniofacial health, the review offers a systematic approach to identifying and treating oral manifestations in IBD.

Diagnosis of Oral Manifestations

Diagnosis of EIMs typically require an oral cavity examination. Authors recommend an oral cavity examination at the time of IBD diagnosis, then annually thereafter. Investigators endorsed a systematic approach that covers the lip mucosa, buccal mucosa, gingiva, the palatal mucosa and oropharynx, the tongue dorsum, tongue ventrum, floor of the mouth, and the teeth. The most common sites of involvement are the lips, buccal mucosa, and gingiva; the most common manifestations include erythema, ulceration, and “cobblestoning”, where certain cells bulge from the mucosal surface in a patterned appearance. The authors provided detailed descriptions of manifestations for the following oral sites:

  1. Lips – Manifestations of the lips may present as mucosal cobblestoning, erythema, fissures, pustules, and ulcerations. Cheilitis may also be present, possibly at the corners of the mouth. Severe, hemorrhagic ulcers of the lips may be an indicator of a severe drug reaction, such as erythema multiforme or Stevens-Johnson Syndrome.
  2. Tongue and Oropharynx – Tongue atrophy, glossitis, and ulcers may be a manifestation of underlying IBD. Additionally, nutritional deficiency secondary to IBD can cause pallor, glossitis, and taste alterations. Certain IBD medications, particularly antitumor necrosis factors, thiopurines, and sulfasalazine, are also associated with oral manifestations, including lichenoid mucositis.
  3. Buccal and Palatal Mucosa – Manifestations in these areas include deep linear ulcerations, fistulae of the vestibular mucosa, erythema, edema, mucosal cobblestoning, and mucosal tags. Aphthous ulcers may also develop secondary to active IBD, nutritional deficiency, or certain medications. Lichenoid ulcerations can also occur on the buccal mucosa.
  4. Gingiva – Erythema, edema, and pustules of the gums may develop in patients with pediatric IBD. Manifestations of the gingiva are often well-managed by typical dental care, including dental plaque removal and periodontal therapy. Other secondary causes of gingival manifestations include vitamin deficiencies and treatment with cyclosporine.
  5. Salivary Glands – Patients with IBD may experience dry mouth as a result of dehydration, IBD medications, or anxiety. Salivary gland manifestations present as inflammation of the sublingual and/or submandibular duct openings.

Management of Oral Manifestations

Referral to an oral medical specialist is recommended, which allows for a timely and appropriate diagnosis. Investigators described treatment methods for oral EIMs based on primary driving factors:


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(1) EIMs Caused by Active Underlying IBD – These manifestations include orofacial Crohn disease (CD), oral aphthous ulcerations, and pyostomatitis vegetans. Oral CD is observed in 5 to 15% of pediatric patients with CD and is diagnosed by punch biopsy. Typically, oral lesions will improve as the active underlying disease is managed. In cases where lesions do not resolve with systemic IBD therapy, topical corticosteroids are recommended, delivered either by gel or mouthwash. In severe cases which impede eating or drinking, systemic corticosteroids may be required.

Oral aphthous ulcerations refer to well-defined ulcers with surrounding erythematosus “halos”. While more common in CD, these ulcers may also occur in ulcerative colitis (UC). Unlike orofacial CD, oral aphthous ulcers do not typically resolve by controlling the underlying IBD. Instead, topical or systemic corticosteroids are recommended to reduce lesion pain and size.

Pyostomatitis vegetans, another EIM associated with active disease, is seen frequently in both UC and CD. Pyostomatitis vegetans presents as multiple white or yellow pustules/ulcers that may form fissures. Lesions often resolve with control of the underlying disease. However, lesions that do not respond to typical IBD treatment may benefit from topical corticosteroids.

(2) Oral EIMs Caused by IBD Medication – Unless reactions are severe, authors recommend against cessation of IBD treatment after development of oral EIMs. Instead, topical corticosteroids are considered first-line treatment. Patients who do not respond to topical therapy may require systemic corticosteroids or intralesion steroid therapy. Severe reactions, such as erythema multiforme and Stevens–Johnson syndrome, necessitate the interruption of IBD therapy. Treatment for these reactions typically includes both topical and systemic steroids.

(3) Oral EIMs Caused by Nutritional Deficiency – Nutritional deficiencies are commonly reported in children with IBD and may cause cheilitis, glossitis, stomatitis, and ulcers. Zinc deficiency may be present in up to 15% of pediatric patients with IBD; other common deficiencies include vitamin B6, vitamin B12, vitamin K, folic acid, and iron. Authors recommend screening for vitamin levels and using dietary supplements to correct observed deficiencies.

Conclusions

Oral EIMs are frequently observed in patients with IBD and may substantially reduce quality of life. Systematic, frequent examinations of the oral cavity are necessary to identify and treat lesions early. In addition to active underlying disease, oral EIMs may be the result of nutritional deficiencies or medication reactions. Early referral to an oral medicine specialist is necessary to confirm diagnosis and develop a comprehensive treatment plan.

Reference

Shazib MA, Byrd KM, Gulati AS. Diagnosis and management of oral extraintestinal manifestations of pediatric inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 2022;74(1):7-12. doi: 10.1097/MPG.0000000000003302