White sponge nevus: Difference between revisions
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{{Infobox medical condition | |||
| name = White sponge nevus | |||
| image = [[File:Autosomal_dominant_-_en.svg|200px]] | |||
| caption = [[Autosomal dominant]] pattern of inheritance | |||
| synonyms = Cannon's disease, Familial white folded dysplasia | |||
| field = [[Oral medicine]] | |||
| symptoms = White, spongy, thickened patches on the [[oral mucosa]] | |||
| complications = None | |||
| onset = Birth or early childhood | |||
| duration = Lifelong | |||
| causes = [[Genetic mutation]] in [[KRT4]] or [[KRT13]] | |||
| risks = [[Family history]] | |||
| diagnosis = [[Clinical examination]], [[biopsy]], [[genetic testing]] | |||
| differential = [[Leukoplakia]], [[Oral lichen planus]], [[Candidiasis]] | |||
| treatment = Usually not required | |||
| medication = None | |||
| prognosis = Excellent | |||
| frequency = Rare | |||
}} | |||
{{Short description|A genetic condition affecting the mucous membranes}} | {{Short description|A genetic condition affecting the mucous membranes}} | ||
==White sponge nevus== | |||
'''White sponge nevus''' (WSN) is a rare genetic disorder characterized by the presence of white, spongy plaques on the mucous membranes, primarily affecting the [[oral cavity]]. It is a benign condition that typically presents in childhood or adolescence. | '''White sponge nevus''' (WSN) is a rare genetic disorder characterized by the presence of white, spongy plaques on the mucous membranes, primarily affecting the [[oral cavity]]. It is a benign condition that typically presents in childhood or adolescence. | ||
==Etiology== | ==Etiology== | ||
White sponge nevus is caused by mutations in the [[keratin]] genes, specifically [[KRT4]] and [[KRT13]], which are responsible for the structural integrity of epithelial cells in the mucous membranes. The condition follows an [[autosomal dominant]] pattern of inheritance, meaning that a single copy of the mutated gene from an affected parent can cause the disorder in offspring. | White sponge nevus is caused by mutations in the [[keratin]] genes, specifically [[KRT4]] and [[KRT13]], which are responsible for the structural integrity of epithelial cells in the mucous membranes. The condition follows an [[autosomal dominant]] pattern of inheritance, meaning that a single copy of the mutated gene from an affected parent can cause the disorder in offspring. | ||
==Clinical presentation== | ==Clinical presentation== | ||
The most common manifestation of white sponge nevus is the appearance of white, thickened, and spongy plaques on the [[buccal mucosa]], although other areas such as the [[gingiva]], [[tongue]], and [[floor of the mouth]] may also be involved. These lesions are usually asymptomatic, but some patients may experience mild irritation or discomfort. | The most common manifestation of white sponge nevus is the appearance of white, thickened, and spongy plaques on the [[buccal mucosa]], although other areas such as the [[gingiva]], [[tongue]], and [[floor of the mouth]] may also be involved. These lesions are usually asymptomatic, but some patients may experience mild irritation or discomfort. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of white sponge nevus is primarily clinical, based on the characteristic appearance of the lesions. A [[biopsy]] may be performed to confirm the diagnosis, revealing hyperparakeratosis and acanthosis of the epithelium. Genetic testing can identify mutations in the KRT4 or KRT13 genes, providing a definitive diagnosis. | Diagnosis of white sponge nevus is primarily clinical, based on the characteristic appearance of the lesions. A [[biopsy]] may be performed to confirm the diagnosis, revealing hyperparakeratosis and acanthosis of the epithelium. Genetic testing can identify mutations in the KRT4 or KRT13 genes, providing a definitive diagnosis. | ||
==Management== | ==Management== | ||
There is no specific treatment required for white sponge nevus, as it is a benign condition. Management focuses on reassurance and monitoring for any changes in the lesions. In cases where the lesions cause significant discomfort, topical treatments or surgical removal may be considered. | There is no specific treatment required for white sponge nevus, as it is a benign condition. Management focuses on reassurance and monitoring for any changes in the lesions. In cases where the lesions cause significant discomfort, topical treatments or surgical removal may be considered. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis for individuals with white sponge nevus is excellent, as the condition does not predispose to malignancy or other serious complications. The lesions typically persist throughout life but do not usually cause significant health issues. | The prognosis for individuals with white sponge nevus is excellent, as the condition does not predispose to malignancy or other serious complications. The lesions typically persist throughout life but do not usually cause significant health issues. | ||
==See also== | |||
== | |||
* [[Oral mucosa]] | * [[Oral mucosa]] | ||
* [[Genetic disorder]] | * [[Genetic disorder]] | ||
* [[Keratin]] | * [[Keratin]] | ||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
Revision as of 19:18, 8 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| White sponge nevus | |
|---|---|
| |
| Synonyms | Cannon's disease, Familial white folded dysplasia |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | White, spongy, thickened patches on the oral mucosa |
| Complications | None |
| Onset | Birth or early childhood |
| Duration | Lifelong |
| Types | N/A |
| Causes | Genetic mutation in KRT4 or KRT13 |
| Risks | Family history |
| Diagnosis | Clinical examination, biopsy, genetic testing |
| Differential diagnosis | Leukoplakia, Oral lichen planus, Candidiasis |
| Prevention | N/A |
| Treatment | Usually not required |
| Medication | None |
| Prognosis | Excellent |
| Frequency | Rare |
| Deaths | N/A |
A genetic condition affecting the mucous membranes
White sponge nevus
White sponge nevus (WSN) is a rare genetic disorder characterized by the presence of white, spongy plaques on the mucous membranes, primarily affecting the oral cavity. It is a benign condition that typically presents in childhood or adolescence.
Etiology
White sponge nevus is caused by mutations in the keratin genes, specifically KRT4 and KRT13, which are responsible for the structural integrity of epithelial cells in the mucous membranes. The condition follows an autosomal dominant pattern of inheritance, meaning that a single copy of the mutated gene from an affected parent can cause the disorder in offspring.
Clinical presentation
The most common manifestation of white sponge nevus is the appearance of white, thickened, and spongy plaques on the buccal mucosa, although other areas such as the gingiva, tongue, and floor of the mouth may also be involved. These lesions are usually asymptomatic, but some patients may experience mild irritation or discomfort.
Diagnosis
Diagnosis of white sponge nevus is primarily clinical, based on the characteristic appearance of the lesions. A biopsy may be performed to confirm the diagnosis, revealing hyperparakeratosis and acanthosis of the epithelium. Genetic testing can identify mutations in the KRT4 or KRT13 genes, providing a definitive diagnosis.
Management
There is no specific treatment required for white sponge nevus, as it is a benign condition. Management focuses on reassurance and monitoring for any changes in the lesions. In cases where the lesions cause significant discomfort, topical treatments or surgical removal may be considered.
Prognosis
The prognosis for individuals with white sponge nevus is excellent, as the condition does not predispose to malignancy or other serious complications. The lesions typically persist throughout life but do not usually cause significant health issues.
