To report rare findings of oral and periodontal manifestations in a patient with Epidermal nevus syndrome (ENS).

The ENS describes the rare association of an epidermal nevus with abnormalities of central nervous system, ocular and skeletal abnormalities. Reports of oral involvement have been few. Also, most of the intraoral lesions have been reported in patients with nevi that do not fulfill the criteria for the diagnosis of ENS.

This report describes a case of ENS that, in addition to cutaneous manifestations showed skeletal involvement and intraoral manifestations such as the extension of the nevi on the face intraorally involving the labial mucosa, hypoplasia, hypodontia of teeth and severe periodontal destruction.

Patients with extensive epidermal nevi and systemic abnormalities should be suspected of having the ENS. Evaluation and management of patients with ENS requires a multidisciplinary team approach involving the dermatologist, pediatrician, ophthalmologist, neurologist, genetist, plastic surgeon and orthopedic services. Although uncommonly described in association with ENS, significant intraoral lesions do occur. Periodontal manifestations as in our patient, which to our knowledge has not been described in association with ENS so far, may also be present.

Alteration of the response of periodontal tissues to dental plaque in the presence of certain systemic diseases has been reported, but not in association with ENS. Severe periodontal destruction due to exaggerated response to dental plaque was seen in the present case. Hence, emphasis on oral hygiene maintenance in such patients is essential. Patients with ENS must be evaluated periodically as they show a persistent predisposition for the development of tumors

Baliga V, Gopinath VPK, Baliga S, Ramachandra SS, Jithendra KD, Shetty S. Intraoral Manifestations in a Patient with Epidermal Nevus Syndrome . J Contemp Dent Pract 2013;14(4):762-765.