The incidence and distribution of oral lesions in patients with Darier's disease.

Darier's disease is an uncommon autosomal dominant genodermatosis that may be accompanied by oral lesions. The incidence and distribution of oral manifestations was studied in 24 patients with cutaneous Darier's disease. Oral lesions comprising whitish papules of varying severity were present in 12 patients, with the palate being the most common site affected, followed by gingiva, buccal mucosa and tongue. Generally, the severity of the oral lesions paralleled that of the skin condition. Seven patients (30%), six of whom also had oral mucosal involvement, reported intermittent parotid swelling. Parotid sialograms of two patients revealed strictures in the main ducts, suggesting an obstructive aetiology.