Monilethrix is an autosomal dominant hair disease that results in short, fragile, broken hair that appears beaded. It comes from the Latin word for necklace (monile) and the Greek word for hair (thrix).


The presentation may be of alopecia. Individuals vary in severity of symptoms. Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.


The genes KRTHB1 KRTHB3 or KRTHB6 that are mutated in this condition code for type II hair cortex keratins.


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