White sponge nevus in a patient with EEC syndrome

Dalben, G.S.; Cursino, Helize A; Barbosa, Bruno Aiello; Costa, Beatríz; Consolaro, Alberto

doi:10.5070/d3482528nt

Cited by 6 publications

(2 citation statements)

References 13 publications

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“…This rare disorder is characterized by abnormalities of the hands, feet, skin, hair, and teeth, including cleft lip and palate [11]. Considering the low prevalence rates of both disorders [12], coexistence could be coincidental. However, three cases have reported an association between WSN and EEC [13,14].…”

Section: Discussionmentioning

confidence: 99%

“…Thus, EEC syndrome presenting with ectrodactyly and with intraoral white plaques that are confirmed by histopathology to be WSN could be linked to a genetic alteration [ 14 ]. Future studies should investigate molecular biology techniques to identify relevant interactions between chromosomal regions 7q11.2-q21.3, which are involved in EEC syndrome, and 12q13 and 17q21, which are related to mucosa-specific keratins [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

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A Case Report on Familial White Sponge Nevus in Saudi Arabia

Akeel¹,

AlFarabi²,

Binsaad³

et al. 2022

Cureus

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Case Report on Familial White Sponge Nevus in Saudi Arabia

Akeel¹,

AlFarabi²,

Binsaad³

et al. 2022

Cureus

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Cleft Palate, with a Peculiar Limb Deformity

El-Darouti

2012

Challenging Cases in Dermatology

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Displasias ectodérmicas: revisión clínica y molecular

García-Martín

Hernández‐Martín

Torrelo

2013

Actas Dermo-Sifiliográficas

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The ectodermal dysplasias are a large group of hereditary disorders characterized by alterations of structures of ectodermal origin. Although some syndromes can have specific features, many of them share common clinical characteristics. Two main groups of ectodermal dysplasias can be distinguished. One group is characterized by aplasia or hypoplasia of ectodermal tissues, which fail to develop and differentiate because of a lack of reciprocal signaling between ectoderm and mesoderm, the other has palmoplantar keratoderma as its most striking feature, with additional manifestations when other highly specialized epithelia are also involved. In recent decades, the genes responsible for at least 30 different types of ectodermal dysplasia have been identified, throwing light on the pathogenic mechanisms involved and their correlation with clinical findings.

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White sponge nevus in a patient with EEC syndrome

Cited by 6 publications

References 13 publications

A Case Report on Familial White Sponge Nevus in Saudi Arabia

A Case Report on Familial White Sponge Nevus in Saudi Arabia

Cleft Palate, with a Peculiar Limb Deformity

Displasias ectodérmicas: revisión clínica y molecular

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