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Pathology · patient case with 2 questions

Patient with oral ulceration and joint and node involvement

Patient: Adult presenting with recurrent oral ulceration

Chief complaint: Painful recurrent mouth ulcers.

Background: Reports joint swelling affecting the knees.

Current findings: Enlargement of the knees. Tender submandibular and anterior and posterior cervical lymphadenopathy.

Question 1

Which diagnosis best fits this combination of findings?

  1. Systemic lupus erythematosus
  2. Crohn disease
  3. Behçet syndrome
  4. Reactive arthritis
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Correct answer: C. Behçet syndrome

  • A. Lupus produces oral lesions and arthritis, but typically with a malar rash, photosensitivity and positive ANA.
  • B. Crohn disease also produces oral ulceration and arthritis, but characteristically with cobblestoned mucosa, mucosal tags and gastrointestinal symptoms.
  • C. BEHÇET SYNDROME is a systemic vasculitis defined by recurrent oral ulceration together with genital ulceration, ocular inflammation (uveitis) and skin lesions, and it commonly involves the joints. The oral ulcers are indistinguishable from major aphthae.
  • D. Reactive arthritis gives the arthritis–urethritis–conjunctivitis triad with painless oral lesions.

Note: The source annotation links this to Sutton disease, the term for major aphthous ulceration — which is the morphology of the oral lesions in Behçet syndrome.

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Question 2

A different patient presents with oral ulceration, and biopsy of the large intestine shows fissured, ulcerated mucosa with a cobblestone appearance. What would biopsy of the oral lesion show?

  1. Non-caseating granulomas
  2. Caseating granulomas
  3. Tzanck cells
  4. An undefined non-specific ulcer
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Correct answer: A. Non-caseating granulomas

  • A. CORRECT. This is CROHN DISEASE. The defining histological feature, in both the gut and the oral lesions, is the NON-CASEATING granuloma. Orally this produces mucosal tags, cobblestoning, linear fissured ulcers in the vestibule and diffuse lip swelling.
  • B. CASEATING granulomas indicate TUBERCULOSIS. The distinction between caseating and non-caseating is the discriminator here.
  • C. Tzanck cells are acantholytic keratinocytes seen in pemphigus vulgaris and in herpetic infection.
  • D. A non-specific ulcer would not explain the systemic picture, and the granuloma is the diagnostic finding.

Note: Non-caseating = Crohn disease, sarcoidosis, orofacial granulomatosis. Caseating = tuberculosis.

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