White sponge nevus
White sponge nevus | |
---|---|
Other names | White sponge naevus, Cannon's disease, Hereditary leukokeratosis of mucosa, White sponge nevus of Cannon, Familial white folded dysplasia,[1][2] or Oral epithelial nevus[3] |
White sponge nevus has an autosomal dominant pattern of inheritance. | |
Specialty | Oral medicine |
White sponge nevus (WSN) is an
keratinization
of the mucosa. This results in lesions which are thick, white and velvety on the inside of the cheeks within the mouth. Usually, these lesions are present from birth or develop during childhood. The condition is entirely harmless, and no treatment is required.
Signs and symptoms
It presents itself in the
alveolar ridge, floor of the mouth, ventral surface of the tongue or soft palate. The gingival margin and dorsum of the tongue are almost never affected. Less commonly, sites outside the mouth are affected, including the nasal, esophageal, laryngeal, anal and genital mucosae.[5] It usually is present from birth, or develops during childhood. Rarely, the lesions may develop during adolescence. Apart from the appearance of the affected areas, there are usually no other signs or symptoms.[5]
Pathophysiology
WSN is caused by a mutation of the
17q21-q22.[8] The condition is inherited in an autosomal dominant manner.[4] This indicates that the defective gene responsible for a disorder is located on an autosome (chromosomes 12 and 17 are autosomes), and only one copy of the defective gene is sufficient to cause the disorder, when inherited from a parent who has the disorder.[citation needed
]
Diagnosis
Differential diagnosis
It is often mistaken for leukoplakia.[9]
Classification
The
skin condition,[10] or more precisely as a genodermatosis (a genetically determined skin disorder).[5]
Treatment
There is no treatment, but because this is a benign condition with no serious clinical complications, prognosis is excellent.[citation needed]
See also
- Oral melanosis
- List of cutaneous conditions caused by mutations in keratins
- Hereditary benign intraepithelial dyskeratosis
References
- ^ Online Mendelian Inheritance in Man (OMIM): 193900
- ISBN 978-1-4160-2999-1.
- ISBN 978-0192628947.
- ^ S2CID 12340591.
- ^ ISBN 978-0721690032.)
{{cite book}}
: CS1 maint: multiple names: authors list (link - PMID 16858417.
- ^ Online Mendelian Inheritance in Man (OMIM): 123940
- ^ Online Mendelian Inheritance in Man (OMIM): 148065
- PMID 27865803.
- ISBN 978-0-7216-2921-6.