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CASE REPORT

Pediatric Dermatology 1–3, 2012

White Sponge Nevus: Clinical Suspicion


and Diagnosis
Murat Songu, M.D.,* Hamit Adibelli, M.D.,* and Gulden Diniz, M.D. 
Departments of *Otorhinolaryngology, and  Pathology, Izmir Dr. Behçet Uz Children’s Hospital, Izmir, Turkey

Abstract: White sponge nevus is a rare, autosomal-dominant disorder


that affects the noncornified stratified squamous epithelia. Clinically, the
presence of white, spongy plaques mostly in the buccal, labial, and gingival
mucosa and the floor of the mouth characterize the lesions. The differential
diagnosis of the lesion may be difficult and it is best diagnosed by biopsy.
We report a case of white sponge nevus in the oral cavity of a 16-year-old boy
and review of the literature.

White sponge nevus (WSN) is an uncommon disease chief complaint of white lesions in the oral cavity and lip
that Hyde first described in 1909, but Cannon (1) coined present since birth. His parents denied presence of a
the term in 1935. This entity is also known by other similar condition in immediate family members or any
names: Cannon’s disease, familial white folded dysplasia, similar lesions elsewhere on his body. Lesions never
hereditary leukokeratosis, white gingivostomatitis, and changed despite numerous interventions such as vita-
exfoliative leukoedema (2,3). mins, oral nystatin suspension, and long-term penicillin
The disorder may be detected in early childhood. The prophylaxis.
lesions are asymptomatic and are discovered inciden- On clinical examination, there were bilateral, sym-
tally. The involved mucosa is white or greyish, thickened, metrical white plaques and patches on the buccal and
folded, and spongy. Lesions are usually misdiagnosed as labial mucosa that could not be removed (Figs. 1 and
candidiasis in children, and the true nature of the disease 2A–C). The margins were well defined, and no lymph
is discovered when antifungal therapy fails. Correct nodes were palpable. Oral hygiene was adequate, and
diagnosis of WSN, which is a benign condition, should oral examination was normal. On histopathologic eval-
be established because other possible ‘‘white’’ lesions uation, thickened epidermis and vacuolization in kerat-
could have malignant potential. We herein present a case inocytes were noted (Fig. 3). Underlying connective
of WSN of the oral cavity in a patient with no history of tissue was normal in appearance. Based on clinical data
familial involvement and a review of the literature. and histopathologic findings, the lesion was consistent
with WSN. The lesions were resistant to tetracycline and
azithromycin treatments. Given that WSN can mimic
CASE REPORT
malignant lesions in the oral cavity, a biopsy and correct
A 16-year-old boy without significant medical history diagnosis is necessary to exclude other concerning
was referred to the outpatient clinic of our hospital with a lesions. Six-month follow-up was recommended.

Address correspondence to Murat Songu, M.D., Department


of Otorhinolaryngology, Dr. Behcet Uz Children’s Hospital, Izmir,
Turkey, or e-mail: songumurat@yahoo.com.

DOI: 10.1111/j.1525-1470.2011.01414.x

Ó 2012 Wiley Periodicals, Inc. 1


2 Pediatric Dermatology 2012

membrane is intact, and the inflammation in the con-


nective tissue generally is slight, if present. Human pap-
illoma virus 16 homologous DNA sequences in the
biopsy specimen of oral WSN were detected using
Southern blot hybridization in one report (6). This con-
dition is mostly attributed to a defect in the normal
keratinization (keratin 4 and keratin 13, which are spe-
cifically expressed in the spinous cell layer of the oral
mucosa) (5,7,8).
Although typically present at birth or in early
childhood, lesions of WSN occasionally may develop in
adolescence. The presence of bilateral, asymptomatic,
thickened, white, corrugated or velvety, soft, ‘‘spongy’’
plaques characterize WSN of the oral cavity. The surface
of the plaque is thick and folded and may peel away from
the underlying tissue. The buccal mucosa is the most
frequent site of involvement, followed by the labial
and gingival mucosa and the floor of the mouth (9).
Extra-oral mucosal sites, such as the nasal, esophageal,
Figure 1. White spongy plaques on the lower lip near the
laryngeal, and anogenital mucosa, appear to be less
commissure bilaterally. commonly involved. Patients are usually asymptomatic
(4,10). The condition may involve the entire oral mucosa,
leaving little normal mucosa visible, or may be distrib-
uted unilaterally as discrete white patches.
DISCUSSION
The recognition of this disorder is important because
White sponge nevus is an inherited disorder exhibiting it should be differentiated from more serious, potentially
autosomal-dominant transmission of variable pene- premalignant lesions. The differential diagnosis of WSN
trance; hence familial reports are not common, similar to includes oral lesions of leukoplakia, chemical burns,
the present case. Neither sex nor racial predilection exists trauma, syphilis, tobacco, and betel nut use. WSN may
(4). WSN has been listed as a rare disorder, with a also be confused with candidiasis; fungal examination,
prevalence rate of less than 1 in 200,000 (5). the histology of biopsy specimens, and unresponsiveness
White spongy plaques that predominantly affect to antifungal agents will be the differentiating factors.
noncornifying stratified squamous epithelia characterize Cheek-biting, lichen planus, and lupus erythematosus
the condition. Histological finding suggests thickening should also be excluded. Lesions of pachyonychia con-
and vacuolization of the spinous layer, with extensive genita, hereditary benign intraepithelial dyskeratosis,
hyperparakeratosis and acanthosis. The basement Darier’s disease, and dyskeratosis congenita may

A B C

Figure 2. (A) White spongy plaques on the lingual mucosa. (B) White spongy plaques on the right buccal mucosa. (C) White
spongy plaques on the left buccal mucosa.
Songu et al: White Sponge Nevus 3

because many other possible ‘‘white’’ lesions could have


malignant potential.

REFERENCES
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lected cases to a limited extent. None of the treatment sponge naevus successfully treated with topical tetra-
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