White Sponge Nevusaka: Cannon's disease or familial white folded mucosal dysplasia

Brittney ShortDate: 11/09/2010

Patient: Silas Carter

Age: 24

Gender: male

Height: 6’0”

Weight: 175

Vital Signs: HR: 70 bmpRespiratory rate: 15 bpmBlood Pressure: 125/86Temperature: 98.5 °F

Chiefcomplaint

The color and appearance is not pleasant, but there is no pain.

MedicalAlert

none

Name of Syndrome:

White Sponge Nevus

Cause of Syndrome - Medical History (if applicable)

White Sponge Nevus is an autosomal-dominant inheritance. The cause is a mutation in the mucosal

keratin 4 or keratin 13 genes. Since it is inherited, it can be present at birth or at puberty.

It is a benign, uncommon, and predominantly affects non-keratinized stratified-squamous epithelia

Orofacial Clinical Features

It presents in the mouth, most frequently as a thick bilateral white plaque with a spongy texture, usually on the buccal mucosa, but

sometimes on the labial mucosa, alveolar ridge or floor of the mouth. The gingival margin and

dorsum of the tongue are almost never affected.

Although this condition is perfectly benign, it is often mistaken for leukoplakia

Age/race/sex predilections and Systemic Clinical Features:

White Sponge Nevus can effect men OR women of every race. If a parent has the disease, their

child has a 50:50 chance of getting inheriting White Sponge Nevus.

Radiographic Features of this Syndrome

none

Special considerations in Treatment of this patient? How is it Diagnosed?How common is it? Is it a horse or zebra?

- White Sponge Nevus is very uncommon. -It is definitely a zebra, and it is often misdiagnosed. It is often thought to be thrush (oral candidiasis), cheek biting, lichen planus, lupus erythematosus, hereditary benign intraepithelial dyskeratosis, tobacco-induced keratotic lesions, pachyonychia congenita, and keratosis follicularis.-It is diagnosed by a biopsy of the lesion(s).-The histopathology of white sponge naevus is very characteristic and in particular shows extensive areas of large clear skin cells in the epidermis.

There is no treatment for White Sponge Nevus. Usually, when doctors first see the characteristics, they try an anti-fungal, but this condition will not respond to such treatment.

At times, the rare case of a plaque which extends onto the lip vermilion and is surgically removed for aesthetic reasons.

Pictures of Disease or Syndrome

Intraoral Pictures

List Sources and References (This should be more than your text book!)

"White Sponge Nevus." Dermatology Online Journal. Web. 03 Nov. 2010. <http://dermatology.cdlib.org/145/nyu/cases/112106_3.html>.

"White Sponge Nevus." Dermatology Online Journal. Web. 03 Nov. 2010. <http://dermatology.cdlib.org/145/nyu/cases/112106_3.html>.

Ibsen, Olga A. C., and Joan Andersen. Phelan. Oral Pathology for the Dental Hygienist. St. Louis, MO: Saunders/Elsevier, 2009. Print.