Ulceration…It’s Not Always What It Seems

Background
Behçet’s is a rare, inflammatory vasculitic disorder that typically presents with oral and genital ulceration. Ulcers are similar in appearance to those seen in recurrent aphthous stomatitis. Erythema Multiforme (EM) is an immune-mediated, mucocutaneous condition with characteristic ‘target lesions’ that can affect mucous membranes. There are many triggers for EM, including infections such as HSV and medications.
Case Description
A 28-year-old male was under long-term care of Rheumatology with a presumed diagnosis of Behçet’s. Concerns were raised regarding worsening oral symptoms including flares of widespread oral ulceration. A referral to Oral Medicine was made for assessment of these ulcers. The patient describes recurrent oral ulcers (since age thirteen) that involve lips, tongue, buccal mucosa, and palate. Additionally, he notes that approximately every eighteen months he develops a very severe flare of oral ulceration with lip crusting. The severity of these flares have led to difficulty eating and drinking, and weight loss.
Outcome
Referral to Oral Medicine led to the additional diagnosis of recurrent Erythema Multiforme (EM), on top of known Behçet’s-related oral ulcers. Identification
of concurrent EM altered the Rheumatology treatment plan, which had been to consider immunosuppression for worsening Behçet’s symptoms. Instead, a trial of Colchicine for the recurrent aphthous ulcers was commenced, and twice daily Acyclovir as prophylactic treatment for recurrent EM.
Discussion
Behçet’s can be a difficult condition to diagnose1; other potential diagnoses for varying presentations of oral ulceration should be explored. More than one condition can confound a diagnosis, and so a detailed ulcer history is essential in ascertaining aetiology. Shared care and close working relationships between associated specialties can help to enhance patient management and share expertise. In this case, it avoided need for immunosuppression and allowed the patient to better understand his two separate diagnoses.
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(1) Jawad ASM, Petrushkin H. Challenges in the diagnosis of Behçet’s disease. Int J Rheum
Dis. 2024 Jan;27(1):e14914. doi: 10.1111/1756-185X.14914. Epub 2023 Sep 15. PMID:
37712444.