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Program

Internal Medicine

Training Level

Resident PGY 2

Institution

Henry Ford Hospital

Abstract

Introduction: Oral manifestations have been reported in crohn’s disease (CD). The reported prevalence of these manifestations varies widely between studies, and range between 0.5%-50% in adults and 10%-80% in pediatrics. Oral manifestations of CD may coincide with the intestinal manifestations, or less commonly precede them. Aphthous ulcers, angular cheilitis, swelling of the lips and gingiva, cobblestoning of the mucosa, deep linear ulcers and periodontal disease have been reported in the literature. Case report: An 18-year-old female with no significant history presented to her primary care physician with recurrent lip swelling and dryness. She was diagnosed initially with angioedema without urticaria. History of an environmental exposure was inconsistent and testing for hereditary angioedema, including C1 esterase and C4 levels were unremarkable. She was referred to a dermatologist who diagnosed her with peri-oral dermatitis. Treatment with antihistamines, topical steroids, antifungals, antibiotics and petroleum jelly-based products were all unsuccessful. She was treated with oral prednisone for a presumed pemphigus vulgaris with a symptomatic improvement, but she did relapse two weeks after weaning steroids. Due to chronicity of symptoms, a lip biopsy was ordered and showed a psoriasiform dermatitis with a granulomatous inflammation. Direct immunofluorescent testing was unremarkable. These results were concerning for CD versus cheilitis granulomatosa. By that time, she denied gastrointestinal symptoms, weight loss or family history of inflammatory bowel disease. Further testing revealed a normal ferritin, vitamin B12, folate, antineutrophil cytoplasmic antibodies, and QuantiFERON-TB. She was referred to gastroenterology for further evaluation. An esophagogastroduodenoscopy showed a normal esophagus, stomach and examined duodenum. Biopsies from stomach and duodenum were unremarkable. A colonoscopy showed a normal examined perianal area, colon and terminal ileum. Biopsies from the left colon showed a focal active colitis, foci of neutrophilic cryptitis and focal epithelioid granuloma without dysplasia. Stains for acid fast bacilli and fungi were negative. Biopsies from the right colon and terminal ileum were unremarkable. A magnetic resonance enterography showed no evidence of an active small bowel CD. Given the early onset and wide spread distribution, the decision was to start adalimumab 40 mg subcutaneously every 2 weeks. Her oral disease improved significantly. A repeated colonoscopy with biopsies was unremarkable. Conclusion: The recognition of oral manifestations can constitute an important clue for diagnosis and management of CD, especially that an isolated oral disease is uncommon as a first presentation of the disease. Infections, nutritional deficiencies and medication side effects are important to consider as differential diagnosis.

Publication Date

5-2019

Chronic Peri-Oral Dermatitis as the First Manifestations of Crohn's Disease

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