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Background: Keratins are a family of highly homologous proteins expressed as pairs of acidic and basic forms which make intermediate filaments in epithelial cells. Keratin 13 (CK13) is the major acidic keratin, which together with K4, its basic partner, is expressed in the suprabasal layers of non-cornified stratified epithelia (1). White sponge nevus (WSN) is a rare autosomal dominant disorder of non-cornifying squamous epithelial differentiation that presents clinically as bilateral white, soft, thick plaques of the oral mucosa. In WSN, the restriction of lesions to mucosal epithelia and the suprabasal cell histopathology parallels the tissue-specific expression of keratins 4 and 13 in the differentiating cell layers (2).