Pathology · single question

A patient presents with painful oral ulcerations, swollen and painful knees, and enlarged tender submandibular and cervical lymph nodes. Which condition BEST accounts for this combination?

  1. Pemphigus vulgaris
  2. Sjögren syndrome
  3. Behçet's disease
  4. Sickle cell anemia
Show answer and explanation

Correct answer: C. Behçet's disease

  • A. Pemphigus vulgaris causes flaccid bullae and painful erosions of oral mucosa and skin with a positive Nikolsky sign, but does not cause arthritis.
  • B. Sjögren syndrome causes dry mouth and dry eyes from lymphocytic destruction of exocrine glands. It may accompany rheumatoid arthritis, but oral ULCERATION is not its hallmark — xerostomia is.
  • C. CORRECT. BEHÇET'S DISEASE is a systemic vasculitis whose defining feature is RECURRENT ORAL ULCERATION (aphthous-like, at least three times yearly), accompanied by some combination of genital ulceration, uveitis and skin lesions. ARTHRITIS is a common systemic manifestation, typically a non-erosive oligoarthritis favoring the KNEES and ankles. The oral-plus-joint pattern described here fits Behçet's directly.
  • D. Sickle cell anemia produces vaso-occlusive pain crises, and the jaws may show a stepladder trabecular pattern and mental nerve neuropathy — but recurrent oral ulceration with knee arthritis is not its presentation.

Note: Behçet's is most prevalent along the historic Silk Road (Turkey, Middle East, East Asia). The pathergy test — a sterile pustule forming at a needle-prick site — supports the diagnosis. Ophthalmology referral is urgent, since uveitis can blind.

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