Description
A 17-year-old boy presented with a 2-month history of painful oral ulcerated lesions and cheilitis (figure 1A–C), with no other symptoms, and no history of trauma or toxic ingestion. His and family medical history were not relevant.
Figure 1.
(A–C) Oral ulcerated lesions and cheilitis.
A biopsy of the oral lesions showed lymphoplasmacytic infiltrate and epithelioid granulomas, compatible with Crohn’s disease (CD) (figure 2).
Figure 2.
Microscopic image (H&E, ×100 magnification) showing epithelioid granulomas in specimen of oral biopsy.
Three months after onset of the oral lesions, the patient started experiencing intermittent abdominal pain and mentioned a change in bowel habits, with loose stools. On examination, he had oral ulcerations, cheilitis and anal skin tags.
Blood analyses showed elevation of erythrocyte sedimentation rate and C reactive protein, and positive anti-Saccharomyces cerevisiae antibody. Upper gastrointestinal endoscopy was normal. Ileocolonoscopy revealed ileocaecal valve deformation, and ileum and colon ulcerations (figure 3A, B). Biopsies of intestinal mucosa showed architectural distortion, inflammatory infiltrate with granulation tissue and epithelioid granulomas, typical features of CD.
Figure 3.
(A and B) Gross endoscopic appearance of terminal ileum showing cobblestone change of the mucosa (fissures and ulcers separate islands of more intact mucosal epithelium) (A), and mucosal ulcerations close to the ileocaecal valve (B).
Four weeks after initiation of oral treatment with 40 mg of prednisolone and 100 mg of azathioprine daily, the patient's oral lesions had significantly improved (figure 4A, B), as had his gastrointestinal symptoms.
Figure 4.
(A and B) Visible improvement of oral ulcerated lesions (A), and cheilitis (B), after initiation of treatment.
CD is a chronic inflammatory process that can involve any region of the alimentary tract.1 Clinical presentation is variable and less typical, and extraintestinal manifestations may precede and dominate the clinical picture.2
CD should be considered in the differential diagnosis of every child with orofacial granulomatosis (OFG). Long-term follow-up is required for patients with OFG as they may develop intestinal CD at a later stage.3
This case illustrates the importance of clinical suspicion for CD in patients, particularly children, with OFG.
Learning points.
Crohn’s disease is a chronic inflammatory process that can involve any part of the gastrointestinal tract.
Less common or extraintestinal manifestations may precede and dominate the clinical picture.
Crohn’s disease should be hypothesised in every child with orofacial granulomatosis.
Acknowledgments
The authors would like to thank Dr Joana Pardal for providing the microscopic image.
Footnotes
Contributors: All the authors contributed to the diagnosis, treatment, literature research and conception of the paper. HA was responsible for the ileocolonoscopy and intestinal biopsies. LSM was responsible for the oral biopsies. All the authors revised the final version of the article.
Competing interests: None declared.
Patient consent: Obtained.
Provenance and peer review: Not commissioned; externally peer reviewed.
References
- 1.Freeman HJ. Natural history and long-term clinical course of Crohn's disease. World J Gastroenterol 2014;20:31–6. 10.3748/wjg.v20.i1.31 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Levine A, Koletzko S, Turner D et al. , European Society of Pediatric Gastroenterology, Hepatology, and Nutrition. ESPGHAN revised porto criteria for the diagnosis of inflammatory bowel disease in children and adolescents. J Pediatr Gastroenterol Nutr 2014;58:795–806. 10.1097/MPG.0000000000000239 [DOI] [PubMed] [Google Scholar]
- 3.Lazzerini M, Bramuzzo M, Ventura A. Association between orofacial granulomatosis and Crohn's disease in children: systematic review. World J Gastroenterol 2014;20:7497–504. 10.3748/wjg.v20.i23.7497 [DOI] [PMC free article] [PubMed] [Google Scholar]