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White sponge nevus

  • Author(s): Dadlani, Chicky
  • Mengden, Stephanie
  • Kerr, A Ross
  • et al.
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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


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 1Figure 2


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.


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.


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