Resident Rounds: Part III - Case Report: Crohn’s Disease Presenting as Granulomatous Cheilitis

February 2016 | Volume 15 | Issue 2 | Features | 251 | Copyright © February 2016

Bryce L. Desmond DO, R. Scott Thomas DO, and Stephanie S. Howerter DO

Western University of Health Sciences – Silver Falls Dermatology, Salem, OR

Abstract
Cutaneous Crohn’s is a rare extra-intestinal manifestation of inflammatory bowel disease seen in a select group of patients, in which cutaneous lesions similar to those of the intestinal illness appear distant from the gastrointestinal tract. Oral findings may be found in up to 60% of patients with extra-intestinal Crohn’s and may appear as the initial symptom underlying disease. We present a case of a 17-year-old male presenting with granulomatous chelitis of the lower lip who was unaware of the underlying diagnosis of Crohn’s disease (CD).

CLINICAL PRESENTATION

The patient is a 17-year-old male who presented to the office with gradual onset of granulomatous cheilitis of the lower lip. He noticed enlargement of his lower lip a few months prior to the appointment, which gradually expanded, and was now at its largest size. He described the lip as non-tender with no symptoms other than occasional dryness and cracking due to expansion. At initial impression, idiopathic granulomatous cheilitis was suspected due to lack of other oral findings and no personal or family history of genodermatoses (ie, Melkerson-Rosenthal, MEN2B, anhidrotic ectodermal dysplasia). The patient also denied any medications or recent illness.
During an extensive review of systems, the patient complained of abdominal pain that would keep him home from school 2 or 3 days per month. The patient attributed this pain to “too much chips and soda” the night before each episode of pain. A referral to gastroenterology was made to check for related causes. An endoscopy and colonoscopy were performed and revealed “ulcerated mucosa in the ileum and transverse colon”. Biopsy of these 2 ulcerations, as well as a random biopsy of the colon, revealed “granulomatous dermatitis with surrounding lymphocytes and plasma cells” consistent with CD. The gastroenterologist decided to start the patient on azathioprine, which theoretically should treat the CD and, indirectly, the granulomatous chelitis. Although the azathioprine was initiated only one month ago, the patient believes he has seen a modest reduction in the size of the lower lip.

DISCUSSION

Some literature has shown that over 40% of patients with CD demonstrate extraintestinal manifestations. Oral findings are the initial presentation in up to 60% of these patients.6 Adolescents and young adults represent the most common patient population to present with oral CD.3 Oral findings can vary; the most specific findings including: persistent cheilitis, oral mucosa cobblestoning, linear and serpiginous ulcerations, mucogingivitis, and polyps. Additionally, some nonspecific findings consist of aphthous ulcers, stomatitis, glossitis, angular cheilitis, and perioral dermatitis.6 Biopsy specimens of these lesions should reveal histologic evidence of granulomatous inflammation. 2
Currently, a cure for CD does not exist and treatment can sometimes run a life-long course. Treatment options depend upon the location and severity of the disease. Single or combination therapy of aminosalicylates, corticosteroids, immunomodulators, and biological agents may be used. Some consider that aminosalicylates have a limited role in CD, and should be reserved for mild cases confined to the colon.5 Systemic corticosteroids can aid in acute flairs but are not used for long-term treatment.4 Older immunomodulators including azathioprine, mercaptopurine, and methotrexate should also be considered. These drugs possess a slower onset of action but have shown efficacy in achieving remission in some patients. Unfortunately, the immunomodulators bring with them possible adverse side effects of bone marrow suppression, liver toxicity, pancreatitis, opportunistic infections, and skin cancer/lymphoma.1 More