Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Leukonychia totalis


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.

Your Question

I have just found out I have this condition. Can you give me some information about it please?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is leukonychia totalis?

Leukonychia totalis is a nail condition characterized by complete whitening of the entire nail plate.[1] It is usually inherited in an autosomal dominant manner. Less commonly, it may be inherited in an autosomal recessive manner, or acquired (not inherited) during a person's lifetime.[2] The inherited forms can be caused by mutations in the PLCD1 gene and generally involve the entire plate of all 20 nails.[3][1] In some cases, leukonychia totalis has been associated with various other abnormalities or syndromes. Treatment may focus on the underlying cause when it is associated with another condition.[1]
Last updated: 2/18/2014

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.[4] 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.[5][4][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

Is leukonychia totalis inherited?

Leukonychia totalis can be inherited in either an autosomal dominant or autosomal recessive manner. It may also occur as part of various underlying conditions or abnormalities, some of which have their own specific genetic cause(s) and inheritance patterns. In some cases, the condition is idiopathic (of unknown cause).[2][5][4]

Autosomal dominant inheritance means that having a change (mutation) in only one copy of the disease-causing gene is enough to cause signs or symptoms. When a person with an autosomal dominant condition has children, each child has a 50% (1 in 2) risk to inherit the mutated copy of the gene.

Autsomal recessive inheritance means that a person must have mutations in both copies of the disease-causing gene to have the condition. Usually, one mutated copy is inherited from each parent, who are each referred to as a carrier.  Carriers of an autosomal recessive condition typically do not have any signs or symptoms.
Last updated: 2/19/2014

How might leukonychia totalis be treated?

There is no universally successful treatment for the whitening of the nails in people with leukonychia totalis.[3] However, if the condition is known to have an underlying cause, treating that cause (when possible) may improve the condition.
Last updated: 2/19/2014

References
  • Howard SR, Siegfried EC. A case of leukonychia. J Pediatr. September, 2013; 163(3):914-915. Accessed 2/18/2014.
  • Yalçin Tüzün, Özge Karakus. Leukonychia. J Turk Acad Dermatol. 2009; 3(1):Accessed 2/18/2014.
  • Marla J. F. O'Neill. NAIL DISORDER, NONSYNDROMIC CONGENITAL, 3; NDNC3. OMIM. August 11, 2011; http://omim.org/entry/151600. Accessed 2/18/2014.
  • 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.
  • 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.