White Sponge Naevus — MFDS Part 1 MCQ
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Correct answer: D — Autosomal dominant; bilateral white corrugated or spongy plaques, usually on the buccal mucosa; benign
White sponge naevus is a rare, usually autosomal dominant mucosal keratin disorder associated with pathogenic variants in KRT4 or KRT13. It typically produces painless, persistent, bilateral white or grey, thickened, folded and spongy plaques, particularly on the buccal mucosa. It is regarded as benign, and asymptomatic disease generally requires reassurance rather than active treatment. Option E describes Peutz–Jeghers syndrome, in which mucocutaneous pigmentation accompanies gastrointestinal hamartomatous polyps. Option A describes oral hairy leukoplakia, an acquired EBV-associated lesion occurring mainly on the lateral tongue in immunosuppressed patients. Painful palatal vesicles suggest recurrent intraoral herpes rather than an inherited leukokeratosis, while a persistent red plaque requires assessment for erythroplakia or another potentially malignant disorder.
Reference: Cai W, Jiang B, Yu F, et al. Current approaches to the diagnosis and treatment of white sponge nevus. Expert Review of Molecular Medicine. 2015;17:e9. https://pubmed.ncbi.nlm.nih.gov/26021387/