Etiology • Hereditary (autosomal-dominant) disorder of keratinizationaffecting nonkeratinizing oral, esophageal, and anogenital mucosal epithelium
• Point mutations in the keratin 4 and/or 13 genes
Clinical Presentation • Asymptomatic • Deeply folded, thickened, white mucosa • Buccal mucosa chiefly affected • No functional impairment • Increased prominence during second decade
Microscopic Findings • Parakeratosis, acanthosis, intracellular edema • Perinuclear condensation of keratin
Diagnosis • Clinical appearance • Family history • Microscopic findings
Differential Diagnosis • Idiopathic leukoplakia • Chemical/thermal burn • Chronic low-grade trauma (morsicatio)
Treatment • None required • No malignant potential
Prognosis • Excellent