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Behcet Disease — SCE Rheumatology MCQ

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EasyVasculitisBehcet DiseaseSCE Rheumatology

A 22-year-old man of Turkish ancestry has recurrent oral and genital aphthous ulcers and several episodes of anterior uveitis. Forty-eight hours after a sterile needle prick, a papulopustular lesion develops at the site. What is the most likely diagnosis?

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Correct answer: CBehçet disease

The diagnosis is Behçet disease. Recurrent oral and genital aphthosis, ocular inflammation and a positive pathergy response form its classic multisystem phenotype. Under the ICBD classification criteria, oral aphthosis, genital aphthosis and ocular lesions each score 2 points, and pathergy scores 1; this patient scores 7, exceeding the threshold of 4. Classification criteria support but do not replace clinical diagnosis. Reactive arthritis can cause oral lesions and uveitis, but genital aphthosis and pathergy are not characteristic, and arthritis usually predominates. Crohn disease can cause oral ulceration and uveitis but would usually have gastrointestinal or perianal disease. Herpes simplex may cause recurrent mucocutaneous ulcers and occasionally uveitis but does not explain sterile pathergy. Genital aphthosis and pathergy are also atypical of SLE.

Reference: International Team for the Revision of the International Criteria for Behçet's Disease. The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. Journal of the European Academy of Dermatology and Venereology. 2014. https://pubmed.ncbi.nlm.nih.gov/23441863/