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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Leukonychia totalis


Other Names for this Disease

  • Hereditary white nails
  • Nail disorder, nonsyndromic congenital, 3
  • NDNC3
  • Porcelain nails
  • Total leukonychia
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Cause

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What causes leukonychia totalis?

Leukonychia totalis (also called total leukonychia) is thought to be due to abnormal keratinization (conversion into keratin) of the nail plate.[1] Keratin is a protein that is a major component of the epidermis (outer layer of skin), hair, nails, and horny tissues.

The condition is usually inherited, following either an autosomal dominant or autosomal recessive inheritance pattern.[2] These inherited forms can be caused by mutations in the PLCD1 gene.[3]

In some cases, leukonychia occurs in association with other underlying abnormalities or syndromes. Conditions that have been reported include palmoplantar keratoderma; certain types of cysts; severe keratosis pilarispili torti; hypotrichosis (lack of hair growth); onychorrhexis (brittle nails); koilonychia (spoon-shaped nails); Bart-Pumphrey syndrome; and Buschkell-Gorlin syndrome, when it occurs with sebaceous cysts and kidney stones.[4][1][2] It has also reportedly been associated with typhoid feverleprosy, cirrhosis, nail biting, trichinosis, and cytotoxic drugs (drugs that are toxic to cells).[2] In a few cases, the cause of leukonychia is unknown (idiopathic).
Last updated: 2/18/2014

References
  1. Lee YB, Kim JE, Park HJ, Cho BK. A case of hereditary leukonychia totalis and partialis. Int J Dermatol. February, 2011; 50(2):233-234. Accessed 2/18/2014.
  2. Yalçin Tüzün, Özge Karakus. Leukonychia. J Turk Acad Dermatol. 2009; 3(1):Accessed 2/18/2014.
  3. Marla J. F. O'Neill. NAIL DISORDER, NONSYNDROMIC CONGENITAL, 3; NDNC3. OMIM. August 11, 2011; http://omim.org/entry/151600. Accessed 2/18/2014.
  4. Balighi K, Moeineddin F, Lajevardi V, Ahmadreza R. A family with leukonychia totalis. Indian J Dermatol. 2010; 55(1):102-104. Accessed 2/18/2014.


Other Names for this Disease
  • Hereditary white nails
  • Nail disorder, nonsyndromic congenital, 3
  • NDNC3
  • Porcelain nails
  • Total leukonychia
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.