White sponge nevus: report of a three-generation family

Introduction. White Sponge Nevus (WSN) is a rare pathology with a pathogenesis on genetic basis, a benign course and a localization affecting the mucosal keratin.WSN is usually a symptomless pathology: when pain is present, some authors reported reduction of symptoms by taking penicillin or oral tetracycline rinses, suggesting that a bacterial overinfection could be at the base of possible painful symptoms.Case Report. We describe 2 patients affected by WSN, father and son: they presented two different oral diseases associated with an infection by Staphylococcus aureus. So, we have performed a careful oral hygiene to reduce infection in the oral cavity. In the following days we prescribed 2 rinses a day with a mouthwash containing chlorhexidine digluconate at two different percentages.Discussion. Early diagnosis of this lesion is important, because it allows us to exclude other more serious diseases. In the most part of cases, WSN requires no treatment because of its benign and asymptomatic behaviour: up to now, no protocol of treatment for this condition was standardized. Even if WSN is a painless condition, sometime a correlated painful symptomatology was reported.Conclusions. In our experience, we have achieved excellent results even with chlorhexidine digluconate rinses, considering that our treated cases were both infected by Staphylococcus aureus.We hypothesize that the corrugated plaques and the altered texture of the mucosa create the right conditions for the colonization and the development of microbial species such as saprophytic bacteria or fungal species.

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“…WSN typically affects several individuals in a same family, further confirming its autosomal dominant heredity 8-9.…”

Section: Introductionmentioning

confidence: 76%

Oral Infection by Staphylococcus Aureus in Patients Affected by White Sponge Nevus: A Description of Two Cases Occurred in the Same Family

Marrelli

Tatullo²,

Dipalma³

et al. 2012

Int. J. Med. Sci.

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“…Of the three large families reported so far, two also showed full penetrance of the disease . In one family, without molecular genetic analysis of the keratin genes to confirm the diagnosis, incomplete penetrance of the WSN phenotype was reported . The absence of both the KRT13 variant as well as any clinical phenotype in the left side of the pedigree supported by haplotype analysis prove that the variant occurred de novo in the grandfather of the index patient.…”

Section: Discussionmentioning

confidence: 93%

“…The majority of WSN are described in case reports, and only a few larger families with WSN have been reported and of these, only two had a KRT13 gene defect [4,8,13]. Here, we report a four-generation family with oral and gynecological manifestations of WSN caused by a novel germ line variant in the KRT13 gene.…”

Section: Introductionmentioning

confidence: 91%

“…Extra-oral lesions are found in other mucosal sites, but are relatively uncommon in the absence of oral manifestations. Extra-oral mucosal sites include the genital, laryngeal, and esophageal mucosa [5][6][7][8][9]. Extra-oral affected sites have been shown to arise only from KRT13 variants.…”

Section: Introductionmentioning

confidence: 99%

“…The histological features of WSN are acanthosis, parakeratosis, cytoplasmic clearing of spinous layer keratinocytes, and characteristic perinuclear eosinophilic condensations that correspond to abnormal aggregation of cytokeratin tonofilaments [11]. The findings through microscopic examination demonstrates that cytology can be very useful in distinguishing WSN from other histopathologically similar lesions [8].…”

Section: Introductionmentioning

confidence: 99%

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