Melkersson-Rosenthal Syndrome — MFDS Part 1 MCQ

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HardOral MedicineMelkersson-Rosenthal SyndromeMFDS Part 1

A 27-year-old patient presents with a third episode of slowly progressive, painless upper-lip swelling. Two years previously, they experienced transient unilateral lower motor neurone facial weakness that was diagnosed as Bell palsy. Examination shows firm, non-pitting lip enlargement and multiple deep grooves across the dorsal tongue. There is no urticaria, mucosal ulceration, otalgia or vesicular eruption. Which diagnosis best integrates these recurrent manifestations?

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Correct answer: C — Melkersson-Rosenthal syndrome

The correct answer is Melkersson-Rosenthal syndrome. Its characteristic manifestations are recurrent or persistent orofacial swelling, relapsing peripheral facial nerve palsy and a fissured tongue (lingua plicata). These features may develop at different times, as in this case, and the complete triad is not always present simultaneously. Lip biopsy may demonstrate non-caseating granulomatous inflammation, but this finding is supportive rather than invariably present. Bell palsy explains isolated peripheral facial weakness but not the lip and tongue findings. Ramsay Hunt syndrome usually includes otalgia and vesicles in the external ear or oral cavity. Angioedema does not account for recurrent facial palsy or lingua plicata. Behçet disease is primarily associated with recurrent oral and genital ulceration, ocular inflammation and other systemic manifestations.

Reference: Wehl G, Rauchenzauner M. A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson-Rosenthal Syndrome. Current Pediatric Reviews. 2018;14(3):196-203. https://pubmed.ncbi.nlm.nih.gov/32161488/