By Gary M. White, MD


Monilethrix is a rare hair shaft defect with autosomal dominant inheritance and variable penetrance.


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.

Differential Diagnosis

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].

Additional Pictures

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Koilonychia in a patient with monilethrix.
Koilonychia in a patient with monilethrix


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