|Year : 2008 | Volume
| Issue : 3 | Page : 125-127
White spongy nevus: A nonhereditary presentation
K Patil, VG Mahima, HS Srikanth
Department of Oral Medicine & Radiology, J.S.S. Dental College & Hospital, Mysore, India
Department of Oral Medicine & Radiology, J.S.S. Dental College & Hospital, Mysore, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
White spongy nevus (WSN) is a rare hereditary dyskeratotic hyperplasia of the mucous membranes. It is an autosomal dominant disorder with variable penetrance. A few cases of WSN occur due to de novo mutations. We report a case of WSN in a 12-year-old female child, with none of her family members having similar lesions.
Keywords: Cannon′s disease, white corrugated mucosa, white spongy nevus
|How to cite this article:|
Patil K, Mahima V G, Srikanth H S. White spongy nevus: A nonhereditary presentation. J Indian Soc Pedod Prev Dent 2008;26:125-7
White spongy nevus (WSN) is a rare genodermatosis. This disease is characterized by an asymptomatic, white folded, spongy mucosal lesion involving the buccal and labial mucosae as well as the floor of the mouth and the soft palate.
| Case Report|| |
A 12-year-old female patient reported for a routine dental checkup [Figure 1]. Her medical and dental histories were insignificant. Diffuse, non-scrapable, raised, white patches were evident on the buccal and labial mucosae, floor of the mouth, lateral tongue borders, ventral tongue, and soft palate [Figure 2],[Figure 3],[Figure 4],[Figure 5],[Figure 6]. The lesions had a wrinkled surface and were spongy on palpation. There were no associated red lesions. The lesions were asymptomatic. The patient stated that she was aware of roughness in her buccal mucosa from as long as she could remember. Patient denied any parafunctional habits. No abnormalities were observed in the scalp hair, nails, palms, soles, and eyes. Other mucosae were found to be free from similar lesions on gynecologic and otolaryngologic examinations.
On observing the oral lesions in the patient, her parents, sibling, and grand parents were called for a detailed oral examination but no such lesions were evident in them.
A smear examination of the buccal mucosa was performed along with an incisional biopsy from the buccal mucosa. Smear examination with PAS stain showed no evidence of fungal hyphae. Under hematoxylin and eosin stain, the sections showed stratified squamous epithelium with hyperparakeratosis, acanthosis, and prominent intracellular edema and vacuolization of the spinous layer. The epithelial cells showed prominent perinuclear condensation of keratin. There was no evidence of dyskeratosis or cellular atypia. The basement membrane was intact and the connective tissue showed normal features [Figure 7] and [Figure 8].
Collectively, the clinical and histopathologic features were suggestive of WSN.
| Discussion|| |
WSN is a rare hereditary dyskeratotic hyperplasia of the mucous membranes. Although Hyde reported the first case of WSN in 1909, a detailed report was published only in 1935 by Cannon. 
It 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, to mention a few. 
WSN is an autosomal dominant disorder with variable penetrance and hence familial reports are not very common, similar to the present case. A few cases of WSN occur due to de novo mutations.  Mutations on the gene K13 are said to be responsible for the development of the disease.  WSN has been listed as a rare disorder, with a prevalence below 1 in 200,000.  Most commonly, lesions appear at birth or in early childhood. Neither gender nor racial predilection exists.  A case of WSN in which human papilloma virus type 16 was demonstrated has been reported in the literature. 
Etiologic factors suggested for WSN include a basic defect in epithelial cell maturation and desquamation, an atypical abundance and aggregation of keratin tonofilaments, and increased intercellular attachment, leading to piling up of surface cells and keratin that would otherwise normally exfoliate. 
WSN is characterized by asymptomatic, white folded, spongy mucosal lesions involving buccal and labial mucosae as well as the floor of the mouth, ventral tongue, and soft palate. The entire oral mucosa can be involved or lesions may appear as discrete white patches unilaterally. The lesions are characteristically spongy on palpation. The gingival margins and dorsum of the tongue are rarely affected. Infrequently, WSN can cause a burning or itchy sensation. Other mucosal sites have also been found to be concomitantly involved such as the anogenital, esophageal, nasal, and laryngeal mucosae; but the skin is spared. ,
Though the lesions of WSN are characteristic they are not pathognomonic. Other lesions that can exhibit a similar clinical appearance include dyskeratosis congenita, pachyonychia congenita, and hereditary benign intraepithelial dyskeratosis (HBID). 
The characteristics of the major differential diagnoses are discussed in [Table 1].
Histopathologically, WSN is characterized by thickened layers of stratified squamous epithelium, extensive hyperparakeratosis, and acanthosis, with edema in the spinous and granulosum layers. The epithelial cells show perinuclear condensation of keratin tonofilaments.
WSN is typically asymptomatic and requires no treatment. It is a benign disorder and does not have the potential to undergo malignant transformation.
Sporadic reports of the use of oral penicillin and topical tetracycline therapy to reduce the prominence of the lesion are available in literature. ,
Nonavailability of a commercial tetracycline mouthwash precluded its usage in the present case and systemic oral penicillin was not found to be effective.
| Conclusion|| |
Many different types of white lesions can occur in the oral mucosa and the appearances of WSN are 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 from the other genodermatosis.
| Acknowledgment|| |
The authors acknowledge Dr Lakshman, General Pathologist, for his contributions.
| References|| |
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|6.||Otobe IF, de Sousa SO, Migliari DA, Matthews RW. Successful treatment with topical tetracycline of oral white spongy nevus occurring in a patient with systemic lupus erythematosus. Int J Dermatol 2006;45:1130-1. [PUBMED] [FULLTEXT]|
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
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