Treatment of monilethrix with oral minoxidil

Fig 1. Patient 1. Dermascopic image of the occipital scalp shows numerous broken hairs. Beaded hairs pathognomic of monilethrix can be seen. INTRODUCTION Monilethrix is an autosomal dominant genodermatosis characterized by hair fragility, keratosis pilaris, and pathognomonic beading of the hair shaft. Hair fragility may lead to hair breakage and the appearance of generalized hair loss. The gene for autosome-dominant monilethrix has been mapped to the epithelial keratin gene cluster on 12q13, and point mutations have been found in the hair cortexespecific keratin genesKRT86,KRT83, and KRT81. Mutations in desmoglein 4 are responsible for an autosomal recessive variant of monilethrix. Monilethrix has considerable interand intrafamilial variations in age of onset, severity, and natural history. Most often, hair is normal at birth and is progressively replaced by short, fragile, brittle hair during the first months of life. Hair breakage secondary to hair fragility may be accompanied by follicular keratosis most commonly on the occiput. Friction from wigs and their adhesives may exacerbate hair loss. Eyebrows, eyelashes, pubic, axillary, and general body hair may be affected along with the scalp. In most patients, the hair loss persists with little change throughout life. Spontaneous improvement or complete recovery has been reported during pregnancy. There is no cure for monilethrix. Reduction in hairdressing trauma may diminish weathering and improve severely affected cases. Two women with monilethrix who responded to 6months of treatment with low-dose oral minoxidil are described.