The authors present several cases of distal onycholysis from the same family. Inheritance is autosomal dominant. The clinical features include a decreased rate of growth of the nail, scleronychia, a straight or concave proximal edge of detachment, palmoplantar hyperhidrosis and marked sensitivity of the fingers to cold. The lesions of the nails are isolated. This is, to our knowledge, only the third report of such an onychopathy.