Uploaded byDr Randy Chance
White sponge nevus
White sponge nevus is a rare, autosomal-dominant disorder affecting the oral mucosa, first described in 1909, characterized by soft, white, corrugated plaques that are painless and non-scrapable. It typically presents in early childhood, has a prevalence of less than 1 in 200,000, and is associated with genetic factors and mutations in keratin genes. Differential diagnoses include various conditions such as oral lichen planus and leukoplakia, with treatment options including antibiotics and antifungals to manage superimposed infections.
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White sponge nevus
- 1. WHITE SPONGE NEVUS DrRandy Chance BMSC BDS
- 2. INTRO • a rare,autosomal-dominant disorder that affects the uncornified stratified squamous epithelia • First described by Hyde in 1909, but Cannon coined the term in 1935 • It’s a type of genodermatoses with problems related to normal epithelial maturation and desquamation- it is unscrapable • No gender or racial predilection • Prevalence rate below 1 in 200,000 • Present from childhood
- 4. OTHER NAMES • Cannon’sdisease • Familial white folded mucosal dysplasia • Hereditary mucosal leukokeratosis • Nevus of Cannon • White folded gingivostomatitis • Exfoliative Leukoedma
- 5. CLINICAL FEATURES • Usuallyoccurs before 20 years of age and often in early childhood • It exhibits no race or gender predilection (or slight female predilection) • Variable penetrance and expressivity • Extra-oral sites such as the mucous membrane of the nasal cavity, esophagus, larynx, rectum and vagina may be involved
- 6. WHITE, SOFT CORRUGATEDAND DIFFUSE PLAQUES IN THE ORAL MUCOSA
- 7. SIGNIFICANT PREDILECTION FORTHE CHEEK MUCOSA Other sites: Ventral tongue Labial mucosa Alveolar ridge Floor of the mouth
- 8. OTHER CLINICAL FEATURES •Painless • Symmetrical • Bilateral • Cannot be scraped off • Differential diagnoses are numerous • No systemic alterations
- 9. PATHOGENESIS • genetic basedpathogenesis, a benign course • HPV 16?? Missense mutations in K4 and K13 genes (mucosal keratins) Interference with intermediate filament assembly Easily damaged intermediate filament Cytokine flooding of underlying basal cells from mild trauma Excessive basal cell proliferation leading to mucosal hyperkeratosis
- 10. DIFFERENTIAL DIAGNOSIS • Hereditarybenign intraepithelial dyskeratosis • Pachyonychia congenital • Dyskeratosis congenital • Candidiasis • Leukoplakia • Cheek biting • Oral submucous fibrosis • Syphillitic mucosal patches • SLE/DLE • Oral Lichen planus
- 11.
- 18. • Prominent hyperparakeratosis •Marked acanthosis • Vacuolization in keratinocytes • Perinuclear eosinophilic condensation of epithelial cells
- 20. PROBLEMS AND TREATMENT •Superimposed infection with Candida and/or Staphylococcus • Tetracycline mouthwash – 0.25% aqueous tetracycline solution, 5ml twice daily. • Amoxycillin PO – initially 250mg three times daily for 4 weeks followed by 250mg once each week to maintain the improvement. • Erythromycin • Antifungal, retinoids, metronidazole, CHX mouthwash
- 21. THE END • Checkout and join my facebook page for regular oral pathology/medicine posts https://www.facebook.com/orpath And my youtube channel for videos of oral pathology and medicine. Youtube channel can be accessed via the facebook page. Thank you.