White sponge nevus in a patient with EEC syndrome

Area of Science:

• Oral Medicine
• Dermatology
• Genetics

Background:

• Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) syndrome is a rare genetic disorder.
• White sponge nevus is a rare benign hereditary intraepithelial bullous disorder of the oral mucosa.

Observation:

• A 10-year-old boy with EEC syndrome presented with oral manifestations including hypodontia, microdontia, enamel hypoplasia, caries, and bilateral buccal mucosal white lesions.
• Histopathological examination of the lesions revealed hyperplastic stratified squamous epithelium with hydropic degeneration and perinuclear eosinophilic condensation.

Findings:

• The patient was diagnosed with white sponge nevus co-occurring with EEC syndrome.
• This represents the first reported case of white sponge nevus in an individual with EEC syndrome.

Implications:

• The co-occurrence of white sponge nevus and EEC syndrome suggests potential shared etiopathogenic factors.
• Further research into the commonalities between these rare conditions may elucidate the underlying mechanisms of white sponge nevus.