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Monilethrix

Monilethrix

Clinical features Monilethrix (beaded hair) is a hair shaft disorder mostly transmitted as an autosomal dominant disorder with incomplete penetrance and variable expressivity.1 It was first described in 1879 by Walter Smith.2 In this disease, the hairs are easily fractured due to areas of narrowing. This results in short, fragile, and easily breakable hairs that result in partial or diffuse alopecia.

The hair shaft resembles a rosary due to the presence of ellipsoidal nodules interrupted by narrow zones. After emerging from the follicle, the hair breaks at the latter sites with resultant hair loss. The alopecia is initially localized to the occipital area, and progressively extends, causing almost total alopecia in those who are severely affected. Involvement of other hair-bearing areas may be seen. The phenotypic expression is variable and the condition sometimes improves with age and pregnancy. Follicular abnormalities range from subtle perifollicular erythema to papules and hyperkeratosis, which are almost invariable concomitant findings.3,4

The differential diagnosis of bubble hair includes pili annulati, fungal infections, and thallium intoxication.7