White sponge nevus

October 28, 2015

BY NANCY W. BURKHART, BSDH, EdD
White sponge nevus is a disease component of a group of inherited diseases
that produce a white, bilateral, keratinized appearance typically appearing
on the buccal mucosa. The term used for this type of genetically induced
lesion within the family of white lesions is genokeratoses. White sponge
nevus (WSN) is an autosomal-dominant disease that is caused by the
mutation of certain keratin genes.
Keratin is the structural protein of epithelial cells. There is a defect in the
normal keratinization (keratin 4 and keratin 13) that is expressed in the
suprabasal keratinocytes of the buccal, nasal, esophageal mucosa, and
anogenital tissue. The disorder is also most commonly known as familial,
white-folded mucosal dysplasia, hereditary leukokeratosis, exfoliative
leukoedema, and as Cannon's disease (described in 1909 by Hyde and given
the name in 1935 by A. B. Cannon).
Although a diagnosis is usually provided in childhood, patients may exhibit a
milder form in their early years, and the disorder may be diagnosed in the
adult much later. Although rare, occurring in less than 1 in 200,000, in recent
years families have become more aware of the genetic link to WSN. With
improved pediatric health histories, the genetic factor is usually recognized
early in life.
Clinical appearance: The patient will exhibit heavy white plaques mainly
on the buccal and labial mucosa that are asymptomatic. The white plaques
may also appear on the floor of the mouth, tongue, and alveolar mucosa with
rare occurrence on the palate. Heavy, thickened tissue may compromise
mastication. Less common areas such as nasal, esophageal, laryngeal, and
anogenital mucosa may be affected.
When viewed microscopically, the tissue exhibits acanthosis or a heavy
thickening of the prickle cell layer of the epithelium. This promotes the thick
white appearance that is very characteristic of WSN. Another feature noted is
the cytoplasmic clearing of the epithelial cells that a pathologist will
recognize.
Differential diagnosis: The clinician would initially rule out:
Leukoplakia
Leukoedema
Lichen planus
Tobacco-related damage to the tissue, such as spit tobacco and betel quid
Chemical burns and temperature-related burns
Candidiasis confirmed through culture (with Candida albicans, the clinician
is able to wipe the white plaque from the tissue, but WSN will not wipe off
with gauze)
Morsicatio buccarum (carefully question the patient regarding this habit)

 Hereditary benign intraepithelial dyskeratosis (Witkop-von Sallman

syndrome)

White Sponge Nevus. Courtesy of Doron Aframian, DMD, PhD.

Other white lesions that may be ruled out after a thorough review of the
health history would include snuff dippers keratosis and oral submucous
fibrosis. Since both of these are tobacco related, careful questioning of the
patient should determine whether these habits would be at the top of your
differential list.
Although the considerations listed above are probably most logical, oral
cancer should never be discounted until proven otherwise. A biopsy is always
needed in unconfirmed cases to rule out malignancy and other previously
mentioned disorders.
An oral medicine perspective: When a definite diagnosis is made of WSN,
the prognosis is excellent and no further treatment is needed for the oral
lesions. Intervention may occur if the tissue is so thickened or dense that it
begins to affect mastication for the patient.

Images courtesy of Murat Songu, MD, department of

otorhinolaryngology, Dr. Behcet Uz Children's Hospital in Izmir,
Turkey.
Some reported treatments within the literature that have shown
improvement in some patients are various vitamins, antihistamines,

chlorhexidine mouth rinses, some antibiotics, and nystatin. However, these

results have been very limited and only beneficial for select patients. Some
researchers believe that the altered tissues make fungus and bacteria more
persistent and harbored within the tissues.
So if the condition is totally benign, why should the dental professional be
concerned about the clinical appearance of white sponge nevus? The
condition is often misdiagnosed, leading to unneeded treatment.
In some patients, the misdiagnosis leads to unnecessary surgery. Children
have been treated for candidiasis with antifungal medications in order to
treat the white plaques. When the areas do not subside, further testing is
usually performed and ultimately discovered to be white sponge nevus.
Reports of older adults are discussed in the literature, and these patients
may not know that the white plaques are white sponge nevus. With adults,
there is often a complaint of the esthetic issues involved with the
appearance of the tongue, lips, or other oral tissue that is visible to others.
There is also a reported altered texture of the tissues as thickness may
increase over time.
Since WSN is usually diagnosed in childhood, the clinician may not even
consider that the adult patient may be exhibiting signs of WSN. A detailed
health history, knowledge of possible etiologies, and attentive listening skills
will assist in the correct course of action for the practitioner.
As always, keep asking good questions and always listen to your
patients. RDH

References
1. DeLong L, Burkhart NW. General and Oral Pathology for the Dental
Hygienist. 2nd Ed. 2013; Wolters Kluwer Health:Lippincott Williams & Wilkins,
Baltimore.
2. Dadlani C, Mengden S, Kerr AR. (2008) White sponge nevus. Dermatology
Online Journal, 14(5). Retrieved from:
http://escholarship.org/uc/item/768308n1
3. Pinto A, Haberland CM, Baker S. Dent Clin North Am 58(20: (2014) 437453.
4. Songu M, Adibelli H, Diniz G. (2012), White sponge nevus: Clinical
suspicion and diagnosis. Pediatr Dermatology, 29(4): 495-497. doi:
10.1111/j.1525-1470.2011.01414.x
5. Vucicevic-Boras V, Cekic-Arambasin A, Bosnjak A. White Sponge Nevus.
Acta Stomat Croat: Case Report 2001;291-292.
NANCY W. BURKHART, BSDH, EdD, is an adjunct associate professor in
the department of periodontics, Baylor College of Dentistry and the Texas A
& M Health Science Center, Dallas. Dr. Burkhart is founder and cohost of the
International Oral Lichen Planus Support Group
(http://bcdwp.web.tamhsc.edu/iolpdallas/) and coauthor of General and Oral
Pathology for the Dental Hygienist. She was a 2006 Crest/ADHA award
winner. She is a 2012 Mentor of Distinction through Philips Oral Healthcare

and PennWell Corp. Her website for seminars on mucosal diseases, oral
cancer, and oral pathology topics is www.nancywburkhart.com. She can be
contacted at nburkhart@bcd.tamhsc.edu.