White sponge nevus
Chicky Dadlani MD, Stephanie Mengden MD, A Ross Kerr DDS MSD
Dermatology Online Journal 14 (5): 16

Department of Dermatology, New York University

Abstract

A 33-year-old-man presented with a 13-year history of asymptomatic, white, folded, soft, poorly-demarcated, diffuse plaques bilaterally on his buccal mucosae and lateral surfaces of his tongue. There is no family history of similar lesions. The physical examination and histopathologic findings were consistent with a diagnosis of white sponge nevus. This rare disorder is typically inherited; however, as in this case, there have been a few other cases reported without a familial background.



Clinical synopsis

A 33-year-old Russian man presented to the Oral Medicine Clinic at Tisch Hospital in June 2006 for the evaluation of white plaques on the tongue and buccal mucosae that had been present for 13 years. He first noted these lesions after receiving several courses of antibiotics for recurrent sinus infections when he was living in Russia. The lesions had remained stable with no new lesions noted over the last 5-7 years. He had received no previous treatment and had applied no topical medications. He denied pain, bleeding, irritation, or dysguesia. Prior to his visit to the Oral Medicine Clinic, he had seen a dentist who referred him to an oral surgeon. He was told he had leukoplakia and there was no further evaluation. Previously, he had consulted a physician in Russia when he first noticed the lesions and was told that the lesions were variations of normal oral mucosa. Medications included antihistamines as needed for rhinitis. He had stopped smoking 10 years ago and occasionally consumes alcohol on weekends. Medical history included recurrent, childhood stomatitis that resolved around age 4. Family history included eczema and psoriasis. No similar oral lesions occurred in any other family members. A punch biopsy was obtained from his left buccal mucosa.


Figure 1 Figure 2

Histopathology

Figure 3

There is parakeratosis, acanthosis, and spongiosis of the mucosal epithelium with blunting of the rete ridges. Vacuolated and dyskeratotic epithelial keratinocytes are present that demonstrate perinuclear eosinophilic condensations.


Comment

White sponge nevus (WSN), which was described by Cannon in 1935, also is known as familial white folded mucosal dysplasia, leukoderma exfoliativum mucosae oris, and hereditary leukokeratosis [1]. It is a benign, uncommon, autosomal dominant disorder that involves a mutation in mucosal keratin that predominantly affects non-keratinized stratified-squamous epithelia [2]. Cases without a familial background have been reported [3]. The onset is in early childhood, with 50 percent of patients being diagnosed before age 20 [4, 5, 6]. White sponge nevus is attributed to a mutation in the helical domain of mucosal specific keratins, K4 and K13 [6]. The mutations are in the form of insertions, deletions, and substitutions that result in abnormal aggregation of tonofilaments and keratin filament instability [7, 8]. Lesions of WSN appear as white-to-gray, diffuse, painless, spongy folded plaques that are typically found on the buccal mucosae. Other common sites include the labial mucosae, tongue, floor of the mouth, and alveolar mucosae. Less frequently, the mucous membranes of the nose, esophagus, genitalia, and rectum are involved [4, 5]. White sponge nevus may be confused with other white lesions of the oral mucosa, which include cheek biting, lichen planus, lupus erythematosus, hereditary benign intraepithelial dyskeratosis, tobacco-induced keratotic lesions, pachyonychia congenita, keratosis follicularis, and candidiasis. Histopathologic findings include parakeratosis, acanthosis with the formation of large, blunt rete ridges, spongiosis, and extensive vacuolation of suprabasal keratinocytes. Dyskeratotic cells exhibit dense peri-and paranuclear eosinophilic condensations, which correspond to tonofilament aggregates. Odland bodies are abundant within keratinocytes, but few are present in the intercellular spaces. This observation suggests a lack of acid phosphatase, which leads to retention rather than normal shedding of superficial cells [6].

No standard treatment for the condition exists although vitamin A [9], antifungal therapy [10], and tretinoin cream have been used [11]. Antibiotic treatment with oral penicillin [12], ampicillin [13], and tetracycline has met with various degrees of success; the use of tetracycline mouthwashes also has been advocated [13, 14, 15]. WSN is not considered to be of bacterial origin; however, since some cases remit with antibiotic therapy, it is possible that infections or inflammation may play a role in the expression of disease [13]. One must consider that the possible beneficial effect of tetracycline is due to modulation of epithelial keratinization [16]. Although WSN is painless, patients may complain of an altered texture of the mucosa or that lesions are not aesthetic. There may be periods of exacerbation and remission; typically, no treatment is sought by patients. Generally, progression of the disorder stops at puberty and there is no malignant transformation [17]. Reassurance is all that is required.

References

1. Cannon AB. White sponge nevus of the mucosa (naevus spongiosus albus mucosae). Arch Dermatol Syphilol 1935; 31: 365

2. Epidermal nevi, neoplasms and cysts. In: Odom RB, et al., eds. Andrews Diseases of the Skin Clinical Dermatology. 9th ed. Philadelphia: Saunders; 2000: 815

3. Part 2: Developmental abnormalities. In: Rushton MA, Cooke BED. Oral Histopathology. Edinburgh: Livingstone, 1963: 140

4. Frithiof L, Banoczy J. White sponge nevus (leukoedema exfoliativum mucosae oris): ultrastructural observations. Oral Surg 1976; 41: 607

5. Jorgensen RJ, Levin S. White sponge nevus. Arch Dermatol 1981; 117: 73

6. Woo S-B. Diseases of the oral mucosa. In: Mckee PH, et al., eds. Pathology of the Skin with Clinical Correlations. 3rd ed. Philadelphia: Elsevier Mosby, 2005:387

7. Rugg EL, et al. A mutation in the mucosal keratin K4 is associated with oral white sponge nevus. Nat Genet 1995;11:450

8. Richard, G, et al. Keratin 13 point mutation underlies the hereditary mucosal epithelial disorder white sponge nevus. Nat Genet 1995;11:453

9. Everett FG, Noyes HJ. White folded gingivostomatitis. J Periodontol 1953;24:32

10. O'Leary PA, et al. White sponge naevus: moniliasis? Arch Dermatol Syphilol 1950;62:608

11. Aloi FG, Moliners A. White sponge nevus with epidermolytic changes. Dermatologica 1988;177:323

12. Alinovi A, et al. White sponge nevus: successful treatment with penicillin. Acta Derm Venereol 1982;63:83

13. McDonagh AJG, et al. White sponge naevus successfully treated with topical tetracycline. Clin Exp Dermatol 1990;15:152

14. Lim J, Ng SK. Oral tetracycline rinse improves symptoms of white sponge nevus. J Am Acad Dermatol 1992;26:1003

15. Lamey PJ, et al. Oral white naevus: responds to antibiotic therapy. Clin Exp Dermatol 1998;23:59

16. Otobe IF, et al. Successful treatment with topical tetracycline of oral white sponge nevus occurring in a patient with systemic lupus erythematosus. Int J Dermatol 2006; 45: 1130

17. Lamey PJ, et al. Oral White sponge naevus: response to antibiotic therapy. Clin Exp Dermatol 1998;23:59

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Title:

White sponge nevus

Journal Issue:

Dermatology Online Journal, 14(5)

Author:

Dadlani, Chicky;
Mengden, Stephanie;
Kerr, A Ross

Publication Date:

2008

Publication Info:

Dermatology Online Journal, UC Davis

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