White sponge nevus in a patient with EEC syndrome
Open accessAbstract
A 10-year-old boy, affected by cleft lip and palate and the Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) syndrome, presented with hypodontia, microdontia, enamel hypoplasia and caries, as well as white macules and plaques on the buccal mucosa bilaterally. Verrucous plaques were noted on the right side and a smooth diffuse white macule was present on the left side. Samples of each were taken by incisional biopsy. Microscopic analysis revealed oral mucosa comprised of hyperplastic stratified squamous epithelium with either hyperparakeratosis or lack of keratinization. There was extensive hydropic degeneration and sparse cells with perinuclear eosinophilic condensation were identified. The underlying fibrous connective tissue exhibited blood vessels with mild subepithelial mononuclear inflammatory infiltrate, bundles of skeletal striated muscle fibers, and areas of hemorrhage at deeper regions. White sponge nevus was diagnosed. To our knowledge, no other case of white sponge nevus has been reported in a patient with EEC syndrome. Considering the low prevalence of this disorder and also of the EEC syndrome, future studies on the etiology of the white sponge nevus might look at the common etiopathogenic aspects of both the white sponge nevus and the EEC syndrome.
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The association between EEC syndrome and WSN is extremely rare and poorly studied.
“The association between EEC syndrome and WSN is extremely rare and poorly studied.”
From Oral white sponge nevus in patients with ectrodactyly-ectodermal dysplasia-cleft syndrome: two patients treated with liquid nitrogen cryotherapy (2026) · cites this paper as [8]
One case of WSN has been reported in a patient with EEC syndrome.
“To our knowledge, one case of WSN has been reported in a patient with EEC syndrome”
From Oral white sponge nevus in patients with ectrodactyly-ectodermal dysplasia-cleft syndrome: two patients treated with liquid nitrogen cryotherapy (2026) · cites this paper as [8]
Cites
Online Mendelian Inheritance in Man.
Oyston, J. (1998) · Anesthesiology · doi:10.1097/00000542-199809000-00060 · Cited by 1,144 · see citing works
A mutation in the mucosal keratin K4 is associated with oral white sponge nevus
Rugg, E. L., McLean, W. H. I., Allison, W. E., Lunny, D. P., Macleod, R. I., Felix, D. H., Lane, E. B., & Munro, C. S. (1995) · Nature Genetics · doi:10.1038/ng1295-450 · Cited by 148 · see citing works
Twenty-four cases of the EEC syndrome: clinical presentation and management.
Buss, P. W., Hughes, H. E., & Clarke, A. (1995) · Journal of Medical Genetics · doi:10.1136/jmg.32.9.716 · Cited by 119 · see citing works
Morsicatio Mucosae Oris—A Chronic Oral Frictional Keratosis, Not a Leukoplakia
Woo, S., & Lin, D. S. (2008) · Journal of Oral and Maxillofacial Surgery · doi:10.1016/j.joms.2008.08.040 · Cited by 88 · see citing works
Jorgenson, R. J. (1981) · Archives of Dermatology · doi:10.1001/archderm.1981.01650020015015 · Cited by 85 · see citing works
A Novel Mutation in the Keratin 13 Gene Causing Oral White Sponge Nevus
Terrinoni, A., Rugg, E. L., Lane, E. B., Melino, G., Felix, D. H., Munro, C. S., & McLean, W. H. I. (2001) · Journal of Dental Research · doi:10.1177/00220345010800031401 · Cited by 47 · see citing works
Annerén, G., Andersson, T., Lindgren, P. G., & Kjartansson, S. (1991) · Clinical Genetics · doi:10.1111/j.1399-0004.1991.tb03093.x · Cited by 43 · see citing works
Terrinoni, A., Candi, E., Oddi, S., Melino, G., Gobello, T., Camaione, D., Mazzanti, C., Zambruno, G., & Knight, R. A. (2000) · Journal of Investigative Dermatology · doi:10.1046/j.1523-1747.2000.00890.x · Cited by 40 · see citing works
White sponge nevus: report of a three-generation family
Júnior, H. M., Pereira, S. M., Rocha, T. M., dos Santos, P. L. A. N., de Paula, A. M. B., & Bonan, P. R. F. (2006) · Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontology · doi:10.1016/j.tripleo.2006.05.029 · Cited by 35 · see citing works
White sponge nevus. Diagnosis by light microscopic and ultrastructural cytology.
Morris, R. W., Gansler, T. S., Rudisill, M. T., & Neville, B. W. (1988) · PubMed · Cited by 30 · see citing works
White Sponge Nevus: Presentation of a New Family
López‐Jornet, P. (2008) · Pediatric Dermatology · doi:10.1111/j.1525-1470.2007.00599.x · Cited by 17 · see citing works
Midface Growth in Patients with Ectrodactyly-Ectodermal Dysplasia-Clefting Syndrome
Birgfeld, C. B., Glick, P. H., Singh, D., LaRossa, D., & Bartlett, S. P. P. (2007) · Plastic & Reconstructive Surgery · doi:10.1097/01.prs.0000263567.62923.fd · Cited by 11 · see citing works
Orthodontic therapy in a patient with white sponge nevus
Quintella, C., Janson, G., de Azevedo, L. R., & Damante, J. H. (2004) · American Journal of Orthodontics and Dentofacial Orthopedics · doi:10.1016/j.ajodo.2003.07.003 · Cited by 11 · see citing works
Lesiones blanquecinas en la mucosa oral
Sambucety, P. S., López, P. M., Prieto, M. Á. R., González, I. R., & Fernández, M. M. (2001) · Anales de Pediatría · doi:10.1016/s1695-4033(01)77653-9 · Cited by 3 · see citing works
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Displasias ectodérmicas: revisión clínica y molecular
García‐Martín, P., Hernández‐Martín, A., & Torrelo, A. (2012) · Actas Dermo-Sifiliográficas · doi:10.1016/j.ad.2012.07.012 · Cited by 41 · see citing works
Ectodermal Dysplasias: A Clinical and Molecular Review
García‐Martín, P., Hernández‐Martín, A., & Torrelo, A. (2013) · Actas Dermo-Sifiliográficas · doi:10.1016/j.adengl.2013.05.001 · Cited by 40 · see citing works
The Pseudolesions of the Oral Mucosa: Differential Diagnosis and Related Systemic Conditions
della Vella, F., Lauritano, D., Lajolo, C., Lucchese, A., Stasio, D. D., Contaldo, M., Serpico, R., & Petruzzi, M. (2019) · Applied Sciences · doi:10.3390/app9122412 · Cited by 34 · see citing works
Current approaches to the diagnosis and treatment of white sponge nevus
Cai, W., Jiang, B., Yu, F., Yang, J., Chen, Z., Liu, J., Wei, R., Zhao, S., Wang, X., & Liu, S. (2015) · Expert Reviews in Molecular Medicine · doi:10.1017/erm.2015.7 · Cited by 33 · see citing works
A Case Report on Familial White Sponge Nevus in Saudi Arabia
Akeel, S. K., AlFarabi, S., Binsaad, S., Almazrooa, S. A., & Binmadi, N. O. (2022) · Cureus · doi:10.7759/cureus.32674 · Cited by 5 · see citing works
Gerlero, P., Lourenço, S. V., & Nico, M. M. S. (2026) · Anais Brasileiros de Dermatologia · doi:10.1016/j.abd.2026.501401
Cleft Palate, with a Peculiar Limb Deformity
El-Darouti, M. A. (2012) · Springer eBooks · doi:10.1007/978-1-4471-4249-2_3
- Authors
- Dalben, G. S., Cursino, H. A., Barbosa, B. A., Costa, B., & Consolaro, A.
- Venue
- Dermatology Online Journal
- Publisher
- California Digital Library (CDL)
- Published
- 2010-05-01
- Volume
- 16
- Issue
- 5
- Pages
- 7-7
- ISSN
- 1087-2108 (ISSN-L)
- OpenAlex
- W1548305490
- Cited by
- 7
- References
- 14
- Citations by year
- 2026: 1 · 2022: 1 · 2019: 1 · 2015: 1 · 2013: 1 · 2012: 2
- FWCI
- 1.11
- Type
- journal article
- Language
- en
- Open access
- green
- escholarship.org
- Licence
- cc-by-nc-nd
- Locations
- Dermatology Online Journal (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by-nc-nd)
- Topics
- Health Sciences › Dentistry › Periodontics › Oral Health Pathology and Treatment
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and rare skin diseases.
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › dental development and anomalies
- Keywords
- white sponge nevus, ectrodactyly-ectodermal dysplasia-clefting syndrome, cleft lip and palate, tooth agenesis, microdontia, enamel hypoplasia, oral lesions, verrucous plaques, hyperparakeratosis, hydropic degeneration, pediatric patients
- MeSH terms
- Abnormalities, Multiple, Child, Cleft Lip, Cleft Palate, Ectodermal Dysplasia, Hand Deformities, Congenital, Humans, Male, Mouth Mucosa, Syndrome, Leukokeratosis, Hereditary Mucosal
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