Hereditary distal onycholysis with scleronychia

Hereditary distal onycholysis is an autosomal dominant nail disease characterized by partial distal onycholysis, reduced nail growth rate, scleronychia, inconstant palmoplantar hyperhidrosis and pronounced sensitivity of the fingers to cold (Bazex et al. 1990). Hereditary distal onycholysis has recently been referred to as "non-syndromal congenital nail disease 5", also known as NDNC5.

In 1966 Schulze described a mother and 2 children with onycholysis of the distal part of the fingernails which were thickened at the same time (scleronychia). Affected were all finger- and toenails. The nail growth was clearly slowed down. Further noticeable was an increased transverse curvature of the nails and missing lunulae. The affected persons (the changes were demonstrably over 5 generations) complained of extreme sensitivity to cold and pressure with paresthesia, but without Raynaud's phenomenon. Furthermore a palmoplantar hyperhidrosis was detectable. Skin, teeth, hair and mucous membranes were normal.

Burg (1975) reported on a family with a patient in whom partial onycholysis was detectable on all fingernails and toenails, with the changes gradually becoming apparent over 10 years. The fingernails were smooth and thickened with increased transverse curvature and separated from the nail bed in the distal half. The nail colour was a waxy dull yellow distally, while proximally the colour was normal, translucent and pale pink. Similar changes were observed in the toenails, which were associated with severe onychodystrophy and subungual hyperkeratosis. Nail growth was normal. No changes were observed in hair, mucous membranes or the rest of the integument. The subject's father, paternal aunt, paternal grandmother and father's cousin had similar changes in their nails.

1. Bazex J et al (1990) Hereditary distal onycholysis--a case report. Clin Exp Derm 15: 146-148
2. Burg G (1975) Onycholysis partialis hereditaria cum skleronychia. dermatologist 26: 386
3. Schulze HD (1966). Hereditary onycholysis partialis with scleronychia. Derm Wschr 152: 766-775.