Darier's disease--oral, general and histopathological features in a 7 year old child

Darier's disease, also known as keratosis follicularis, is a rare autosomal dominant genodermatosis, manifesting clinically as hyperkeratotic, firm papule that predominates in the seborrheic areas and flexures with accompanying nail abnormalities. Heat, sweating, sunlight, and stress are exacerbating factors of the lesion. The oral lesions are asymptomatic and comprise multiple white papules in the buccal mucosa and hard palate, giving a cobblestone appearance, and it is characterized histologically by loss of desmosomal adhesion and abnormal keratinization resulting in mucocutaneous abnormalities. Pruritus, recurrent infections, and malodor can decrease the quality of life. We report a case of a 7-year-old boy with clinical and histological features of Darier's disease.