Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Hale C White sponge nevus. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticwhitespongenevus.html. Accessed June 2nd, 2023.
Definition / general
- Not a melanocytic entity; benign keratinization defect leading to white plaques on mucosa
- Primarily affects nonkeratinized stratified squamous epithelium
- First described by Cannon in 1935 (Arch Dermatol Syphilol 1935;31:365)
Terminology
- Synonyms: familial white folded mucosal dysplasia, leukoderma exfoliativum mucosae oris, hereditary leukokeratosis
Epidemiology
- Rare, typically autosomal dominant disorder due to mutation in mucosal keratins CK4 (Br J Dermatol 2003;148:1125) and CK13 (J Dent Res 2001;80:919)
Sites
- Buccal, gingival, esophageal, rectal or genital mucosal surfaces
Clinical features
- White to gray, diffuse, painless, spongy and folded plaques on buccal mucosa
Case reports
- 33 year old man with buccal lesion, no family history (Dermatol Online J 2008;14:16)
Treatment
- No standard treatment; although reports of tetracycline, chlorhexidine
- Progression usually stops at puberty
- No malignant transformation
Clinical images
Microscopic (histologic) description
- Parakeratosis, acanthosis with formation of large blunt rete ridges and spongiosis
- Extensive vacuolation of suprabasal keratinocytes
- Dyskeratotic cells exhibit dense peri and paranuclear eosinophilic condensations, which correspond to tonofilament aggregates
- Abundant Odland bodies (keratinosome, membrane bound granule in upper stratum spinosum) within keratinocytes but few are present in the intercellular spaces
Differential diagnosis
- Candidiasis
- Hereditary benign intraepithelial dyskeratosis
- Lupus erythematosus
- Morsicatio buccarum: chronic cheek biting
- Morsicatio linguorum: chronic tongue biting
- Mucosal lichen planus
- Oral submucous fibrosis
- Pachyonychia congenita
- Tobacco induced keratotic lesions