Dariers Disease with Oral Manifestations : A Clinical Rarity : Case Report

Darier disease (DD), also known as keratosis folicularis and White disease , is an inherited autosomal dominant genodermatosis. DD often develops in childhood, persists through adolescence and causes small papules predominantly in seborrheic areas such as the face , chest and back. The oral lesions are asymptomatic and comprise multiple white papules in the buccal mucosa and the soft and hard palate , giving a cobblestone appearance. Histologically , DD is characterized by corps ronds and grains in addition to suprabasal cleavage. There are no currently validated curative treatments available for DD, with the majority of cases treated symptomatically. This is a case report of DD with varied manifestations intraorally and extraorally. Diagnosis was established on the basis of clinical , histopathological and dermatological findings. Patient reported to the OPD with a chief complaint of loose teeth. A holistic treatment approach was carried out based on clinical and histological findings. Functional and esthetic demands were met with satisfactory results.