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White Sponge Nevus


What is one type of epithelial genodermatosis of the oral cavity? This is a White Sponge Nevus.
What are some alternative names for White Sponge Nevus? Some alternative names are Cannon Disease and Familial White Folded Dysplasia.
What are some clinical features of White Sponge Nevus? - It is a rare genetic condition as the autosomal dominant trait is caused by mutations in either the keratin 4 or keratin 13 genes. - These lesions may be evident at birth or early in childhood, but sometimes in the second decade.
What does White Sponge Nevus look like clinically? - They are bilateral diffuse, wrinkled, white exophytic plaques. - They occur most commonly on the buccal mucosa. - They are asymptomatic and do not disappear when the cheek is stretched. Some patients report that the white areas may peel off.
What are some other areas where White Sponge Nevus can be found? - They can be found on the ventral tongue, lips, soft palate, oral floor and alveolus. - Other areas include the nasal sinus, esophagus, larynx, and anogenital mucosa.
True or False: There is no gender predilection for White Sponge Nevus and this may be diagnosed as a congenital finding or in infancy, while others are diagnosed in early childhood. True.
What is used to give a diagnosis of White Sponge Nevus? The clinical and family history are important for a presumptive diagnosis. However, an exfoliative cytology is also helpful. Genetic analysis is warranted.
What are some microscopic features of White Sponge Nevus? With a Papanicolaou stain, one finds the condensation of keratin filaments around the keratinocyte nuclei. A biopsy shows hyperparakeratosis and acanthosis of the epithelium.
What are some microscopic features of White Sponge Nevus? (continued) The keratinocytes have perinuclear condensation of keratin monofilaments and intracellular edema.
What treatment is done in the case of White Sponge Nevus? Treatment is not needed with clinical correlation. These lesions are not problematic and remain unchanged after the first few years of proliferation.
What can cause a White Sponge Nevus lesion to become more white and thickened? Smoking or cheek biting habits can make this worse and these habits should be followed closely.
True or False: White Sponge Nevus has a good prognosis with no malignant transformation. True.
What are differential diagnoses for White Sponge Nevus? - Hereditary Benign Intraepithelial Dyskeratosis - Leukodema - Smokeless Tobacco Keratosis - Morsicatio - Candida - Proliferative Verrucous Leukoplakia - Verrucous Carcinoma
Created by: seema.prakash