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      A Case Report on Familial White Sponge Nevus in Saudi Arabia

      case-report
      1 , , 1 , 2 , 3 , 1
      ,
      Cureus
      Cureus
      gene mutations, oral mucosal lesions, hyperkeratosis, genodermatosis, white sponge nevus

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          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          White sponge nevus (WSN) is an uncommon, benign, autosomal dominant disorder that usually appears at birth or in early childhood. It affects males and females equally and is caused by germ line mutations of the keratin genes leading to keratin instability and tonofilament aggregation. The condition causes painless, white, thickened, corrugated plaques to form on the oral mucosa, especially bilaterally on the buccal mucosa. Extra-orally, it occurs most often in the vaginal mucosa, as well as in the nasal and esophageal mucosa. In this report, we describe the case of a healthy 32-year-old Saudi male in Jizan in southern Saudi Arabia whose general dentist referred him to the oral medicine clinic at King Abdulaziz University Hospital, where he was diagnosed with white sponge nevus. The patient reported no medical problems and was a cigarette smoker for more than 10 years. An oral examination revealed white lesions affecting the buccal mucosa bilaterally and the labial mucosa. A biopsy of the buccal mucosa confirmed the diagnosis of white sponge nevus. Laser therapy was suggested for the aesthetic treatment of the lesions. Better awareness of this hereditary condition among dental professionals can help improve timely diagnoses early in life and thus avoid unnecessary or inadequate treatment for this benign condition.

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          Most cited references16

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          The pseudolesions of the oral mucosa: Differential diagnosis and related systemic conditions

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            Genetic and developmental disorders of the oral mucosa: Epidemiology; molecular mechanisms; diagnostic criteria; management.

            A large number of disorders may affect the oral cavity, including genetic diseases, infections, cancers, blood diseases, skin diseases, endocrine and metabolic disorders, autoimmune and rheumatologic diseases, local lesions, to name a few. Oral mucosa shows a considerable variation in its normal structure and a wide range of conditions may affect it. Such conditions are often harmless or minor and could be primary or secondary to systemic disease. Several of them are quite rare and, hence, the diagnosis is not easy. Clinically, lesions may appear as ulcers, discoloration of the oral mucosa and alterations in size and configuration of oral anatomy. Genetic disorders have specific manifestations and can be caused by a derangement of one or more components of the tissue. Many of them follow the skin or systemic signs of the underlying genetic disease, but in a few cases oral signs could be the first manifestation of the disorder. Among them genodermatoses are prominent. They are inherited disorders characterized by a multisystem involvement. This review describes chondro-ectodermal dysplasia, dyskeratosis congenita, Ehlers-Danlos syndrome, hereditary benign intraepithelial dyskeratosis, keratosis follicularis, lipoid proteinosis, multiple hamartoma syndrome, pachyonychia congenita, Peutz-Jeghers syndrome, tuberous sclerosis and white sponge nevus. Other genetic disorders not included in the genodermatosis group and reported in the present review are: acanthosis nigricans, angio-osteo-hypertrophic syndrome, encephalotrigeminal angiomatosis, familial adenomatous polyposis, focal dermal hypoplasia, focal palmoplantar and oral mucosa hyperkeratosis syndrome, gingival fibromatosis, Maffucci's syndrome, neurofibromatosis (type 1) and oro-facial-digital syndrome (type 1). Disorders during embryonic development might lead to a wide range of abnormalities in the oral cavity; some of them are quite common but of negligible concern, whereas others are rare but serious, affecting not only the oral mucosa, but also other structures of the oral cavity (ie palate, tongue and gingiva). Fordyce's granules, leukoedema, cysts of the oral mucosa in newborns, retrocuspid papilla, geographic tongue, fissured tongue, median rhomboid glossitis, hairy tongue, lingual varices and lingual thyroid nodule are described. This review may help dentists, dental hygienists, but also general internists and pediatricians to diagnose different disorders of the oral mucosa, to understand the pathogenesis and to schedule a treatment plan.
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              White sponge naevus: improvement with tetracycline mouth rinse: report of four cases.

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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                18 December 2022
                December 2022
                : 14
                : 12
                : e32674
                Affiliations
                [1 ] Oral and Maxillofacial Pathology, King Abdulaziz University, Jeddah, SAU
                [2 ] Oral and Maxillofacial Pathology, King Abdulaziz University Hospital, Jeddah, SAU
                [3 ] Dentistry, King Abdulaziz University, Jeddah, SAU
                Author notes
                Article
                10.7759/cureus.32674
                9848695
                1618052d-bfff-4ca6-8392-30a5d9176372
                Copyright © 2022, Akeel et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 15 December 2022
                Categories
                Pathology
                Dentistry
                Oral Medicine

                gene mutations,oral mucosal lesions,hyperkeratosis,genodermatosis,white sponge nevus

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