Isolated anonychy-onycholysis L60.8

Authors: Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Martina Bacharach-Buhles

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Last updated on: 29.10.2020

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Synonym(s)

Anonychia-Onycholysis isolated; Nail disorder, nonsyndromic congenital 9; NDNC9; OMIM 614149

Definition
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Although nails appear normal at birth in this autosomal recessive inherited syndrome, dystrophic changes in the nails develop during the first decade of life, leading to onycholysis of the fingernails and anonymity of the toenails. This disorder is called non-syndromic congenital nail disorder-9 (NDNC9).

Etiopathogenesis
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This is based on a mutation of a gene localized on chromosome 17q25.1-17q25.3 (Rafiq MA et al. 2004).

Clinical features
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Rafiq et al. (2004) reported on a consanguine Pakistani family in which a form of hereditary nail dysplasia was detected over 6 generations. The affected persons had normal nails at birth. The onychodystrophy developed at the age of 7 or 8 years and led to an anonymity of the toenails as well as to onycholysis of the fingernails. Further anomalies of the ectodermal appendages were not observed in any of the affected individuals.

Literature
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  1. Rafiq MA et al (2004) Localization of a novel locus for hereditary nail dysplasia to chromosomes 17q25.1-17q25.3 Clin Genet 66: 73-78
  2. Zaias N et al (2015) Finger and toenail onycholysis. J Eur Acad Dermatol Venereol 29:848-853.

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