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Genetic and Rare Diseases Information Center (GARD)

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Monilethrix


Other Names for this Disease

  • Nodose hair
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

What is known about monilethrix?  I was told that there isn't a cure.  Is there any research being done to discover a cure?  What type of treatment is currently available?

Our Answer

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

What is monilethrix?

Monilethrix is a rare condition caused by a defect in the hair shaft resulting in hair which appears dry, dull, and brittle, and which breaks spontaneously or with mild trauma.  The age of onset, severity, and course may vary from person to person.[1]
Last updated: 1/30/2012

What parts of the body does monilethrix affect?

Monilethrix occurs mainly on the scalp, predominantly on the occiput and nape and occasionally on other scalp areas. Scalp involvement can be widespread or localized. Occasionally, the eyelashes, eyebrows, pubic, axillary, and limb hair are involved.[2]
Last updated: 1/30/2012

What are the characteristics of monilethrix?

Monilethrix is characterized by a beaded appearance of the hair due to periodic thinning of the shaft. This condition results in hair fragility and patchy dystrophic alopecia (hair loss). The term monilethrix is derived from the Latin word monile, which means necklace, and the Greek word thrix, which means hair. This term describes the resemblance of the hair to a string of beads or a necklace.[2]
Last updated: 1/30/2012

What causes monilethrix?

The cause of monilethrix remains unclear. To date, whether monilethrix is a disorder of the function or structure of the hair has not been determined. Some genetic studies suggest that monilethrix is likely caused by a mutation (change in a gene) in keratin (a type of protein found in the hair).[2] At least four genes have been found to cause this condition. Autosomal dominant monilethrix is caused by mutations in the hair cortex keratin genes KRT81, KRT83, or KRT86. The autosomal recessive form of monilethrix results from mutations in the desmoglein 4 (DSG4) gene.[3][4]

Last updated: 1/30/2012

Is there treatment available for monilethrix? Is there a cure for the condition?

Unfortunately, the is no cure for monilethrix. Some patients have reported spontaneous improvement, particularly during puberty and pregnancy, but the condition rarely disappears completely.[1][2]

While there is no recognized definitive treatment for monilethrix, oral acitretin and topical 2% minoxidil have shown good clinical and cosmetic results with continued use.[2][5][6] The systemic administration of vitamins, retinoids, griseofulvin, oral contraceptives, steroids, radiation therapy, external desquamative ointments, and steroid preparations have not show impressive results.[2]

Avoiding trauma is perhaps the most effective method of managing monilethrix. This is because from birth, the hair of individuals with monilethrix tends to have an increased susceptibility to weathering and cosmetic damage (e.g., sunlight exposure, dyeing, bleaching, perming, curling). This susceptibility to damage can prevent hair from growing to its maximum length.[2]

Last updated: 3/12/2014

Are there any researchers currently studying ways to treat or cure monilethrix?

Although we are not aware of any researchers currently studying ways to treat or cure monilethrix, we recommend that you periodically check PubMed, a searchable database of medical literature, to locate published articles regarding treatment and a possible cure. Using "monilethrix AND treatment" as your search term should locate articles. To narrow your search, click on the “Limits” tab under the search box and specify your criteria for locating more relevant articles.
Last updated: 3/12/2014

References
  • Kliegman RM, Behrman RE, Jenson HB, Stanton BF. Chapter 661 - Disorders of Hair. Kliegman: Nelson Textbook of Pediatrics, 18th ed. Philadelphia, PA: Saunders, An Imprint of Elsevier; 2007;
  • Schwartz RA, Alexiewicx-Slowinska M. Monilethrix. Medscape Reference. September 12, 2013; http://emedicine.medscape.com/article/1118500-overview#. Accessed 3/12/2014.
  • Monilethrix. Online Mendelian Inheritance in Man (OMIM). May 6, 2010; http://omim.org/entry/158000. Accessed 1/30/2012.
  • Farooq M, Ito M, Naito M, Shimomura Y. A case of monilethrix caused by novel compound heterozygous mutations in the desmoglein 4 (DSG4) gene. Br J Dermatol. 2011; http://www.ncbi.nlm.nih.gov/pubmed/21495994. Accessed 1/30/2012.
  • Karincaoglu Y, Coskun BK, Seyhan ME, Bayram N. Monilethrix: improvement with acitretin. Am J Clin Dermatol. 2005; http://www.ncbi.nlm.nih.gov/pubmed?term=16343029. Accessed 1/31/2012.
  • Rossi A, Iorio A, Scali E, Fortuna MC, Mari E, Palese E, Greco P, Carlesimo M. Monilethrix treated with minoxidil. Int J Immunopathol Pharmacol. 2011; http://www.ncbi.nlm.nih.gov/pubmed?term=21496408. Accessed 1/31/2012.
Other Names for this Disease
  • Nodose hair
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.