JODDD Vol. 3 No. 2 Spring 2009
Dental Research, Dental Clinics, Dental Prospects
White Sponge Nevus: A Case Report
Amirala Aghbali 1 • Firouz Pouralibaba 2* • Hossein Eslami 3 • Farzaneh Pakdel 3 • Zahra Jamali 3
1 Assistant Professor, Department of Oral and Maxillofacial Pathology, Faculty of Dentistry, Tabriz University of Medical Sciences, Tabriz, Iran
2 Assistant Professor, Department of Oral Medicine, Faculty of Dentistry, Tabriz University of Medical Sciences, Tabriz, Iran
3 Post-graduate Student, Department of Oral Medicine, Faculty of Dentistry, Tabriz University of Medical Sciences, Tabriz, Iran
*Corresponding Author; E-mail: firstname.lastname@example.org
Received: 28 February 2009; Accepted: 14 April 2009
J Dent Res Dent Clin Dent Prospect 2009; 3(2):70-72
This article is available from: http://dentistry.tbzmed.ac.ir/joddd
© 2009 The Authors; Tabriz University of Medical Sciences
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which
permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
White sponge nevus (WSN) is a rare hereditary dyskeratotic hyperplasia of mucous membranes. It is an autosomal dominant
disorder with variable penetrance. We report a case of WSN in a healthy 21-year-old male with no history of familial
involvement. A white smooth plaque with no erythema or other structural abnormalities was observed, which confirmed the
diagnosis of WSN histopathologically.
Key words: Dyskeratosis, white lesion, white sponge nevus.
hite sponge nevus (WSN) is a relatively rare
cutaneous and mucosal lesion.1,2,3 Hyde
reported the first case of WSN in 1909 and a detailed
report was published in 1935 by Cannon.4,5
Etiologically, it is a rare developmental anomaly
inherited as an autosomal dominant trait with variable
expressivity and a high degree of penetrance. This
condition is attributed to a defect in the normal
keratinization (keratin 4 and keratin 13, which are
specifically expressed in the spinous cell layer of the
oral mucosa).6-11 This keratotic mucosal alteration
may be seen on vaginal and rectal mucosa but the
great majority of cases involve the oral mucosa.1
A search of dermatological and gynecological
literature revealed very little about WSN in Iran. More
information was available about this lesion in the oral
cavity, which was retrieved from the dermatological
and dental literature.12
Lesions of WSN usually appear at birth or in early
childhood, but sometimes the condition develops
during adolescence. The lesions consist of symmetric,
thickened, white, corrugated or velvety, diffuse
plaques. Buccal mucosa is the most frequently
affected, followed by the labial and gingival mucosa,
and the floor of the mouth. Extra-oral mucosal sites,
such as the nasal, esophageal, laryngeal, and
anogenital mucosa, appear to be less commonly
affected. Patients are usually asymptomatic. The white
color does not diminish when the tissue is stretched in
any mucosal site.1,2,6,13,14
The recognition of this disorder is important in that
it must be differentiated from other congenital or
familial disorders of more widespread clinical
significance. The clinical appearance is so distinctive
that biopsy is usually unnecessary. The microscopic
features of WSN are characteristic but not necessarily
pathognomonic. Prominent hyperparakeratosis and
marked acanthosis with clearing of the cytoplasm of
the cells in the spinous layer are common features;
however, similar microscopic findings may be
associated with leukoedema and hereditary benign
intraepithelial dyskeratosis. In some instances an
eosinophilic condensation is noted in the perinuclear
A Case of White Sponge Nevus 71
JODDD Vol. 3 No. 2 Spring 2009
region of the cells in the superficial layers of the
epithelium, a feature that is unique to WSN.1-5
In this paper, a case of WSN in a healthy white male
with no history of familial involvement is described.
The patient was a 21-year-old Iranian male referred to
the Department of Oral Medicine at Tabriz University
of Medical Sciences Faculty of Dentistry for diagnosis
and management of a “white, itchy spot” on the
buccal mucosa. White bilateral lesion in oral mucosa
was the chief compliant of the patient. The patient
complained of a white lesion which was present since
The patient’s general health was reportedly good.
The patient denied presence of a similar condition in
immediate family members or any similar lesions
elsewhere on his body.
In clinical examination, there were bilateral,
symmetrical white plaques and patches on the buccal
and labial mucosa, which could not be removed
(Figure 1). The plaques were smooth with velvety
texture and irregular, well-defined borders. There was
no elevation or erythema. The margins were clear and
no lymph nodes were noticeable. Oral hygiene was
good and other oral structures were normal in
In histopathologic evaluation, oral mucosa covered
by stratified squamous epithelium revealed prominent
hyperparakeratosis and marked acanthosis with
clearing of the cytoplasm of cells in the spinous layer.
In addition, eosinophilic condensation was noted in
the perinuclear region of the cells in superficial layers
(Figure 2). Underlying connective tissue was normal
in appearance with rare chronic inflammatory cell
Based on clinical data and histopathologic findings,
the lesion was consistent with white sponge nevus.
Because of benign nature of this lesion, no treatment
is necessary and only biopsy and correct diagnosis is
necessary to rule out other similar lesions. Six-month
follow-up was recommended.
WSN is a rare hereditary dyskeratotic hyperplasia of
mucous membranes. This entity is also known by
other names, such as Cannon's disease, familial white
folded hypertrophy of the mucous membranes,
hereditary leukokeratosis, white gingivostomatitis,
and exfoliative leukoedema.1-5 WSN is an autosomal
dominant disorder with variable penetrance and hence
familial reports are not very common, similar to the
present case. WSN has been listed as a rare disorder,
with a prevalence rate below 1 in 200,000.13 Most
commonly, lesions appear at birth or in early
childhood. Neither gender nor racial predilection
exists.14 A case of WSN, in which human papilloma
Figure 1. Clinical views of the lesions.
Figure 2. (a) Histopathologic view of the lesion (×10); (b)
perinuclear condensation of keratin tonofilament
(arrow) (×40) (H&E).
Aghbali et al.72 Download full-text
JODDD Vol. 3 No. 2 Spring 2009
virus type 16 was demonstrated, has been reported in
the literature.4 Many different types of white lesions
can occur in the oral mucosa and the appearance of
WSN is not pathognomonic. There is a need for
precise identification through prompt histopathologic
examination to differentiate this condition from more
serious, potentially premalignant lesions as well as
other genodermatoses such as hereditary benign
epithelial dyskeratosis, lichen planus, lichenoid drug
reaction, lupus erythematosus, cheek chewing and
possibly candidiasis. While some of these lesions are
benign, others are pre-malignant or manifestations of
some systemic diseases. Therefore, early diagnosis of
this benign lesion is important,3 and often, these
lesions need different treatment plans.15-21 In addition,
these lesions reveal different epidemiological patterns
and involve different societies and races. In Northwest
Iran, this condition seems to be rare and no other
similar documented cases are available. In this case,
none of the family members had similar lesions. This
lesion appeared early in life without any reported
changes throughout the patient’s life, but diffuse
spreading of the lesion seems to be an alarming factor.
Biopsy in such cases is necessary for treatment
planning and ruling out of other lesions.
1. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral &
Maxillofacial Pathology, 3rd ed. St. Louis: WB Saunders;
2. Elder DE, Elenitsas R, Johnson BL, Murphy GF. Lever's
Histopathology of the Skin, 9th ed. Philadelphia: Lippincott
Williams & Wilkins; 2005: 715.
3. Regezi JA, Sciubba JJ, Jordan R CK. Oral Pathology, 5th ed.
St. Louis: Saunders Elsevier; 2008: 74-77.
4. Cox MF, Eveson J, Porter SR, Maitland N, Scully C. Human
papillomavirus type 16 DNA in oral white sponge nevus. Oral
Surg Oral Med Oral Pathol 1992;73:476-8.
5. Cannon AB. White spone nevus of the mucosa. Arch Dermat
and Syph 1935; 31:365-70.
6. Jorgenson RJ, Levin LS. White sponge nevus. Arch
7. Búchholz F, Schubert C, Lehmann-Willenbrock E. White
sponge nevus of the vulva. Int J Gynaecol Obstet
8. Nichols G, Cooper P, Underwood P, Greer K. White sponge
nevus. Obstet Gyn 1990;76:545-8.
9. Woo SB. Diseases of the oral mucosa. In: McKee P, Calonje
E, Granter S, eds. Pathology of the Skin, With Clinical
Correlations, 3rd ed. Philadelphia: Mosby; 2005: 387.
10. Dadlani C, Mengden S, Kerr AR. White sponge nevus.
Dermatol Online J 2008;14:16.
11. Rugg EL, McLean WH, Allison WE, Lunny DP, Macleod RI,
Felix DH, et al. A mutation in the mucosal keratin K4 is
associated with oral white sponge nevus. Nat Genet
12. Jahanbani J, Sandvik L, Lyberg T, Ahlfors E. Evaluation of
oral mucosal lesions in 598 referred Iranian patients. Open
Dent J 2009;3:42-7.
13. Shibuya Y, Zhang J, Yokoo S, Umeda M, Komori T.
Constitutional mutation of keratin 13 gene in familial white
sponge nevus. Oral Surg Oral Med Oral Pathol Oral Radiol
Endod 2003; 96:561-5.
14. Hernandez-Martin A, Fernandez-Lopez E, de Unamuno P,
Armijo M. Diffuse whitening of the oral mucosa in a child.
Pediatr Dermato1997; 44:316-20.
15. Lamey PJ, Bolas A, Napier SS, Darwazeh AM, Macdonald
DG. Oral white sponge navus: response to antibiotic therapy.
Clin Exp Dermatol 1998; 23:59-63.
16. Everett FG, Noyes HJ. White folded gingivostomatitis. J
Periodontol 1953; 24:32.
17. O'Leary PA, Montgomery H, Brunsting LA, Kierland RR.
White sponge nevus: moniliasis? Arch Dermatol Syphilol
18. Aloi FG, Moliners A. White sponge nevus with epidermolytic
changes. Dermatologica 1988; 177:323-6.
19. Alinovi A, Benoldi D, Pezzarossa E. White sponge nevus:
successful treatment with penicillin. Acta Derm Venereo
20. McDonagh AJ, Gawkrodger DJ, Walker AE. White sponge
naevus successfully treated with topical tetracycline. Clin Exp
21. Lim J, Ng SK. Oral tetracycline rinse improves symptoms of
white sponge nevus. J Am Acad Dermatol 1992; 26:1003-5.
The article entitled “Accuracy of digital subtraction radiography in combination with a contrast media in
assessment of proximal caries depth” which appeared in J Dent Res Dent Clin Dent Prospect 2008; 2(3):77-81
incorrectly listed the second author’s affiliation as Department of Oral and Maxillofacial Radiology, Shahid
Beheshti University of Medical Sciences. The correct affiliation of Sara Ehsani is Dental Research Center of
Shahid Beheshti University of Medical Sciences.