Leukonychia totalis is a rare disorder of the nails, where all the nails of the fingers and toes appear strikingly white. The disease may be congenital, or may be acquired later in life secondary to severe systemic illness. The whitening of the nails may sometimes be associated with camptodactyly, hammer toes, gingivitis, hyperkeratosis, deafness, skin cysts, syndactyly of fingers, joint contractures and/or arthrogryposis. Treatment is not required unless for the associated symptoms. Patients need to be reassured that the whitening is not due to any nutritional defect.
Mutations in the PLCD1 (Phospholipase C Delta 1) gene is responsible for causing leukonychia totalis and/or partialis. Familial cases of the disease have been shown to follow an autosomal dominant mode of inheritance. An autosomal recessive mode of inheritance has also been implied [see Epidemiology].