Molecular Pathogenesis and Complications Associated with Keratosis Follicularis: A Clinical Review

Abstract

Keratosis follicularis or Darier s disease (DD) is a rare autosomal dominant disorder characterized by the appearance of multiple scaly papules affecting seborrheic areas. It is a multisystem disorder that occurs in the first or second decade of life, and extends beyond cutaneous involvement. It has been reported to be associated with various basal cell carcinoma (BCC) and other skin cancers, nail changes, ocular/mucosal manifestations and neuropsychiatric disorders. Additionally, individuals with DD have a greater risk of being diagnosed with Type 1 Diabetes Mellitus as well as disease-specific risk of heart failure. The goal of this review is to explore the molecular pathogenesis of keratosis follicularis, and to investigate the extent and severity of various complications associated with this condition. Furthermore, dermatologic practice recommendations will be reviewed for the management of DD.