The lesion is white or greyish, thickened, folded and. White sponge nevus wsn in the oral mucosa is a rare autosomal dominant genetic disease. White sponge nevus wsn is one of a number of white lesions of the oral mucosa. White sponge nevus wsn is an interesting hereditary oral mucosal disorder that commonly manifests as bilaterally symmetrical, thickened. It has also been called congenital leukokeratosis, mucosa oris, hereditary leukokeratosis, white folded gingivostomatitis, oral epithelial naevus and oral white. It is characterized by asymptomatic white plaques affecting mainly the oral mucosa. White sponge naevus mendelian inheritance in man mim number 193900 is a rare genetic condition that causes white spongy lesions of the mucous membranes, most commonly the mouth. Jun 05, 2009 white sponge nevus wsn is a rare hereditary dyskeratotic hyperplasia of mucous membranes. The mucous membranes of the nose, esophagus, genitalia and rectum may also be involved. Symptoms of white sponge nevus of cannon including 9 medical symptoms and signs of white sponge nevus of cannon, alternative diagnoses, misdiagnosis, and correct diagnosis for white sponge nevus of cannon signs or white sponge nevus of cannon symptoms. Ce 110 a guide to clinical differential diagnosis of oral.
White sponge nevus an overview sciencedirect topics. Wsns occur on the mucosa of the nasopharynx, mouth, esophagus, larynx, vagina, and anus, wsns may respond to penicillin. Jun 11, 2015 white sponge nevus wsn is a rare periodontal hereditary disease that was first described by hyde and coined by cannon. White sponge nevus wsn is a rare autosomal dominant disorder of the noncornifying squamous epithelia. White sponge nevus registered dental hygienist rdh. It is an autosomal dominant disorder with variable. For a discussion of genetic heterogeneity of white sponge nevus, see wsn1 193900. White sponge nevus wsn is a rare periodontal hereditary disease that was first described by hyde and coined by cannon.
White sponge nevus presenting as genital lesions in a 28year. It is characterized by white, thickened, folded and spongy lesions of the oral mucosa, although the esopha. A case report of white sponge nevus of oral cavity international. Herein, we present a new case of oral white sponge nevus in a 17yearold iranian. This results in lesions which are thick, white and velvety on the inside of the. Although the white color in leukoplakia is a result of hyperkeratosis or acanthosis, similarly appearing white lesions that are caused by reactive keratosis smokers keratosis or frictional keratoses e. It is characterized by white, thickened, folded and spongy lesions of the oral mucosa, although the esophageal, laryngeal, nasal and anogenital mucosa might also be affected. Careful clinical and histopathological examination is indicated to exclude other more serious conditions presenting as oral white lesions. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. The term used for this type of genetically induced lesion within the family of white lesions is genokeratoses.
Bilateral, white, keratotic plaques, most commonly on buccal mucosal surface and sometimes on labial, lingual, and other mucosal sites. Jun 01, 2014 white sponge nevus wsn is a rare and autosomal dominant genetic disease in which the oral mucosa is white or greyish, thickened, folded, and spongy. Pdf white sponge nevus wsn is a rare hereditary dyskeratotic hyperplasia of mucous membranes. The white sponge nevus is thought to be a familial form of keratosis of the mucous membranes. Oral disease 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. The nevi are most commonly found on the moist lining of the mouth oral mucosa, especially on the inside of the cheeks buccal mucosa. Lokasi yang paling umum pada epitel mukosa tidak berkeratin seperti di mukosa pipi, bilateral dan selanjutnya di mukosa bibir, lingir alveolar dan dasar mulut. Leukoedema may be confused with leukoplakia, dariers disease, white sponge nevus, pachyonychia congenita, or candidal infection. Dec 17, 2011 white sponge nevus almost always presents during childhood and there is no gender predilection.
White sponge nevus is a condition characterized by the formation of white patches of tissue called nevi singular. It is an autosomal dominant disorder of wide variability and high penetrance. Buy this article and get unlimited access and a printable pdf. White sponge nevus wsn, is an autosomal dominant condition of the oral mucosa the mucous membrane lining of the mouth. White sponge nevus wsn is a rare autosomal dominant disorder which was first described by hyde in 1909, but the term was coined in 1935 by cannon. May 29, 2015 white sponge nevus wsn in the oral mucosa is a rare autosomal dominant genetic disease. Oral medicine white sponge nevus eeport of a case herbert f. White sponge nevus is a rare autosomal dominant genetic disease of the oral mucosa. It presents in the mouth, most frequently as a thick bilateral white plaque with a spongy texture, usually on the buccal mucosa, but sometimes on. It is characterized by white, thickened, folded and spongy lesions of the oral mucosa, although the esophageal, laryngeal, nasal and anogenital mucosa might also be. White sponge nevus wsn is an autosomaldominant disease that. Pdf oral infection by staphylococcus aureus in patients.
This results in lesions which are thick, white and velvety on the inside of the cheeks within the mouth. Familial case of oral white sponge nevus a rare hereditary. White sponge nevus wsn is a rare autosomal dominant disorder of noncornifying squamous epithelial differentiation that presents clinically. Esophageal white sponge nevus associated with severe dysphagia and odynophagia. White sponge nevus wsn is a rare pathology with a pathogenesis on genetic basis, a benign course and a localization affecting the mucosal keratin. White sponge nevus is a rare genodermatosis first described by cannon in 1935 fig. White sponge nevus wsn is a rare and autosomal dominant genetic disease in which the oral mucosa is white or greyish, thickened, folded, and spongy. The mucous membranes of the nose, esophagus, genitalia and. White sponge nevus is a developmental and a rare cutaneous and mucosal lesion. In recent years, new cases of wsn and associated mutations have been reported. It is caused by a mutations in certain genes coding for keratin, which causes a defect in the normal process of keratinization of the mucosa. The differential diagnosis of the lesion may be difficult and it is best diagnosed by biopsy. The clinical hallmarks are white, soft, spongy, thickened plaques on both sides of the oral mucosa and tongue. White sponge nevus definition of white sponge nevus by the.
The lesions may be diffuse or patchy, and are usually asymptomatic. The involved mucosa is white or greyish, thickened, folded and spongy. It is an autosomal dominant disorder with variable penetrance. White sponge nevus definition of white sponge nevus by. White sponge nevus almost always presents during childhood and there is no gender predilection. The white sponge nevus is a hereditary leukokerotosis localized preferably in the oral mucosa, but may simultaneously manifest itself in other regions, e. Pdf on jul 11, 2016, sanjeeta n and others published white sponge nevus. The characteristic histopathologic features are epithelial thickening, parakeratosis, and vacuolization of the suprabasal layer of oral epithelial keratinocytes. The onset is usually during early infancy, often before 20 years, and there is no gender predilection. The onset is early in life, and both sexes are affected equally. Pdf white sponge nevus in a patient with eec syndrome. Clinically, the presence of white, spongy plaques mostly in the buccal, labial, and gingival mucosa and the floor of the mouth characterize the lesions. Leukoplakia could be classified as mucosal disease, and also as a premalignant condition.
Oral infection by staphylococcus aureus in patients affected. White sponge nevus wsn is a relatively rare cutaneous and mucosal lesion. It presents in the mouth, most frequently as a thick bilateral white plaque with a spongy texture, usually on the buccal mucosa, but sometimes on the labial mucosa, alveolar ridge or floor of the mouth. Case report oral infection by staphylococcus aureus in. White sponge nevus presenting as genital lesions in a 28yearold female a case is presented of white plaques occurring predominantly on the vulvar mucosa of a 28yearold female diagnosed as white sponge nevus wsn. 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. Keratin point mutation underlies the hereditary mucosal. White surface lesions of oral mucosa surface lesions of oral mucosa that appear white, tan, or light yellow are divided into three groups based on their clinical features. White sponge nevus wsn is an interesting hereditary oral mucosal disorder that commonly manifests as bilaterally symmetrical, thickened white, corrugated or.
White sponge nevus is a rare benign autosomal dominant disorder with variable penetrance. White sponge nevus is a rare, benign, autosomal dominant leukokeratosis that predominantly affects the oral mucosa. Intro a rare, autosomaldominant disorder that affects the uncornified stratified squamous epithelia first described by hyde in 1909, but cannon coined the term in 1935 its a type of genodermatoses with problems related to normal epithelial maturation and desquamation it is unscrapable no gender or racial. White sponge nevus wsn is a rare hereditary dyskeratotic hyperplasia of mucous membranes. Definisi white sponge nevus merupakan lesi keratotik pada mukosa yang tanpa gejala, putih, berkerut dan seperti busa, seringkali lesinya memperlihatkan pola gelombang yang simetris. White sponge nevus is a benign heritable process that is caused by any one of a variety of mutations of the genes responsible for production of either keratin. We report a case of wsn in a healthy 21yearold male with no history of familial involvement. White lesions due to accumulation of necrotic debris on the mucosal. Intro a rare, autosomaldominant disorder that affects the uncornified stratified squamous epithelia first described by hyde in 1909, but cannon coined the term in 1935 its a type of genodermatoses with problems related to normal epithelial maturation and desquamation it is unscrapable no gender or. Wsn is a rare, autosomal dominant disorder involving mucous membranes. White sponge nevus nichols 1991 international journal. The genes associated with wsn include mutant cytokeratin keratin 4 krt 4 and keratin krt. Leukoedema is a white or whitishgray edematous lesion of the buccal and labial oral mucosa. A case is presented of white sponge nevus involving the vaginal, labial, and oral mucosae of a 34yearold woman.
Pdf introduction white sponge nevus wsn is an uncommon white lesion mainly affecting the oral mucosa. Less frequently, it affects extraoral sites including the vulvovaginal mucosa. White sponge nevus is a disease component of a group of inherited diseases that produce a white, bilateral, keratinized appearance typically appearing on the buccal mucosa. A rare disorder characterized by the presence of soft, white, and spongy plaques in the oral mucosa.
23 752 891 296 515 357 1401 805 724 122 736 195 992 1284 36 361 913 52 333 1518 1001 666 1513 609 904 1460 495 1362 1347 656 1398 1221 611 1 1191 1067 655 623 645 969 1022