What is Darier’s disease?

Darier’s disease, also known as keratosis follicularis and Darier-White disease, is a dominantly inherited disorder of keratinization caused by mutations in the ATP2A2 gene, resulting in dysfunction of intracellular calcium signaling. Typically, the disease presents in the first or second decade of life. Microscopically, it is characterized by distinctive changes of both premature keratinization and acantholysis.