Table of Content


Autumn 2015, Vol. 23 No. 3

Hong Kong J. Dermatol. Venereol. (2015) 23, 126-128

Paediatric Dermatology Column: Case Report

White sponge naevus: recognition of this disorder is important to differentiate from premalignant oral lesions


S Seremet, FS Afsar, H Duran, G Yilmaz, M Ermete


White sponge naevus (WSN) is a rarely seen autosomal-dominant disease. It is characterised by white plaques seen on the buccal, gingival and oral mucosae. It has a prevalence of less than 1/200,000. This entity is also known as Cannon's disease, hereditary leucokeratosis of mucosa, white gingivostomatitis or exfoliative leucoedema. Given that WSN is a rare disease, we present a 16-year-old boy with WSN.


Keywords: Autosomal-dominant, oral mucosa, white sponge naevus

關鍵詞: 常染色體顯性、口腔黏膜、白色海綿狀痣

Case report

A 16-year-old boy presented to our clinic because of white-coloured plaques with a spongy appearance on the mucosae of both his lips and cheeks (Figure 1). These asymptomatic lesions were present since birth and his brother also had similar lesions. On physical examination; his oral hygiene was good, with no lymphadenopathy or any accompanying nail-related abnormality. Punch biopsy from the patient's mucosa was done with a differential diagnosis of white sponge naevus (WSN), leucoplakia and oral lichen planus. The histopathological examination revealed hyperkeratosis of the epidermis and vacuolisation of the keratinocytes (Figures 2 & 3). Fungal stains for candida infection were performed three times, all of which were negative. On the basis of these clinical and histopathological findings, we made the diagnosis of WSN. The lesions did not respond to treatment with tetracycline or azithromycin; the patient is still being followed up every six months.

Figure 1 White-coloured plaques with a spongy appearance on the mucosa of both his lips and cheeks.

Figure 2 Hyperkeratosis of the epidermis and vacuolisation of the keratinocytes (H&E, 4x).

Figure 3 Transparent cytoplasm and vacuolisation in the keratinocytes (H&E, 20x).


WSN is a disease that involves the stratified, non-cornified flat epithelium. Hyde first described the condition in 1909, but Cannon coined the term in 1935.1 Differentiating this condition with other diagnoses may be difficult.2,3 Its typical phenotype includes widespread, white and spongy plaques that can be seen in the oropharyngeal, nasal, oesophageal and anogenital sites. The most significant and frequently-involved site is the oral mucosa and non-mucosal involvement is not seen. It appears at birth, and is most prominent in adolescence and remains stable throughout life.4,5 Histological findings of the plaques include hyperproliferation, acanthosis and cytoplasmic vacuolisation. A mutation in the K4 and K13 keratin types is responsible for the lesion and tissue irregularity among patients with WSN.4 The lesions are painless and the patients complain about their undesirable cosmetic appearance and symptoms resulting from the changing structure of the mucosa. A detailed history and biopsy are required to establish the diagnosis. The differential diagnosis should include other diseases that cause white lesions such as congenital pachyonychia, hereditary benign intraepithelial dyskeratosis, lichen planus, leucoplakia, chronic candidiasis, tobacco-induced keratotic lesions and chronic cheek biting.1,2 WSN is a congenital benign disorder without any risk of malignant transformation. The recognition of this disorder is important because it should be differentiated from the more serious, potentially premalignant lesions. It does not require any treatment. There have been reports in which systemic antibiotic treatment, especially tetracycline and azithromycin, leading to significant recovery.3,4 In this case, taking the histopathological findings of the white-coloured plaque lesions over the mucosa of the cheeks together with the fact that the occurrence of the lesions for years without developing any symptoms, we diagnosed the patient with WSN.

We presented our case with WSN, which is a rare disorder and should be considered in patients with persistent, asymptomatic white plaques on their mucosa.


1. Cannon AB. White spone nevus of the mucosa. Arch Dermatol and Syph 1935;31:365-70.

2. Hernandez-Martin A, Fernandez-Lopez E, de Unamuno P, Armijo M. Diffuse whitening of the oral mucosa in a child. Pediatr Dermatol 1997;44:316-20.

3. Lamey PJ, Bolas A, Napier SS, Darwazeh AM, Macdonald DG. Oral white sponge nevus: response to antibiotic therapy. Clin Exp Dermatol 1998;23:59-63.

4. Luccese A, Favia G. White sponge naevus with minimal clinical and histological changes: report of three cases. J Oral Pathol Med 2006;35:317-9.

5. deTomas MJ, Bagan JV, Silvestre FJ, Milian MA, Jimenez Y. White sponge nevus: presentation of sixteen cases corresponding to six families. Med Oral 1999;4:494-502.