Darier's Disease Of Moderate Severity With Mucosaland Nail Involvement

ABSTRACT

Darier's disease, also known as keratosis follicularis, dyskeratosis follicularis or Darier-White disease, is a rare, inherited, acantholytic disorder caused by mutation in ATP2A2 gene, which manifests with hyperkeratotic lesions of variable severity. It typically occurs in young age and presents with itching and greasy appearance. The lesions are persistent and disease follows a chronic course with protean prognosis. Extracutaneous involvement may occur in mucosa, nail, eye and salivary gland.We report a case of Darier's disease of teenage onset, and moderate severity with palmoplantar, mucosal and nail involvement.