By Gary M. White, MD
Monilethrix is a rare hair shaft defect with autosomal dominant inheritance and variable penetrance.
- The term monilethrix (necklace hair) is a combination of the Latin monile (necklace) and the Greek thrix (hair).
- Most cases are autosomal dominant.
- The AD inherited form is caused by mutations of the genes encoding type 2 hair keratins, hHb1 and hHb6.
- The AR inherited form has in cases been associated with mutations in desmoglein 4.
- The hair shaft is beaded with resulting fragility of the hair.
- Koilonychia may be associated in some families.
Onset is usually in early childhood with the nape and occiput being most severely affected. In some cases, all body hair may be affected. Keratotic papules may be seen at the follicular opening. Koilonychia may be associated.
See fragile hair.
Unfortunately, there is no cure. Because the hair is fragile, gentle hair care is necessary. Wigs may be needed for older girls and women. Improvement may occur with age. Low dose off-label oral monoxidil (0.25 mg daily) helped grow hair in two patients [JAAD Case Rep. 2016 May; 2(3): 212–215].
Koilonychia in a patient with monilethrix.
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