White sponge nevus: a case report.
ABSTRACT 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.
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ABSTRACT: White sponge nevus (WSN) is a benign hereditary lesion of the mucous membranes. DNA extracted from a biopsy specimen of oral WSN was assayed for the presence of DNA sequences homologous to human papillomavirus (HPV) types 1, 2, 4, 6, 11, 13, 16, and 18 by Southern blot hybridization. Only HPV-16 homologous DNA sequences were detected at a copy number of approximately 200 to 250 genome copies per diploid cell. The viral DNA sequences did not appear to be integrated into the host cell chromosome. The finding of HPV-16 in an inherited lesion such as WSN indicates that caution must be exercised in ascribing a causal association in relation to the demonstration of HPV in other mucosal disorders.Oral Surgery Oral Medicine Oral Pathology 05/1992; 73(4):476-8.
Article: White sponge nevus of the vulva.[Show abstract] [Hide abstract]
ABSTRACT: The white sponge nevus is a hereditary leukokerotosis localized preferably in the oral mucosa, but may simultaneously manifest itself in other regions, e.g. perianally. We report the case of a 18-year-old patient with a primarily and exclusively extraorally located white sponge nevus of the vulva. The differential diagnosis and clinical as well as therapeutical problems of this extremely rare disease are discussed.International Journal of Gynecology & Obstetrics 01/1986; 23(6):505-7. · 1.56 Impact Factor
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ABSTRACT: White sponge nevus (WSN) is a benign autosomal dominant disorder which affects non-cornifying stratified squamous epithelia (MIM 193900) (ref. 1). Phenotypically it presents as white 'spongy' plaques (oral leukokeratoses), most commonly in the mouth but also reported in the esophagus and anogenital mucosa. Histologically, the plaques show evidence of hyperproliferation, acanthosis and tonofilament aggregation. These types of pathogenic changes are characteristic of many of the epidermal keratin disorders. Keratins are expressed in pairs by epithelial cells in a tissue and cell specific manner. The major differentiation specific keratins of the buccal mucosa, nasal, esophageal and anogenital epithelia are K4 and K13 (ref. 7). The tissue distribution and nature of the lesions in patients affected by WSN suggested that mutations in K4 and/or K13 might be responsible for this disorder. We have now confirmed this hypothesis and report here a three base-pair (bp) deletion in the helix initiation peptide of K4 in affected members from two families with this condition.Nature Genetics 01/1996; 11(4):450-2. · 29.65 Impact Factor
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: email@example.com
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
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.
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3. Regezi JA, Sciubba JJ, Jordan R CK. Oral Pathology, 5th ed.
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6. Jorgenson RJ, Levin LS. White sponge nevus. Arch
7. Búchholz F, Schubert C, Lehmann-Willenbrock E. White
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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
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Armijo M. Diffuse whitening of the oral mucosa in a child.
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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.