Monilethrix is associated with follicular keratosis (horny follicular papules).
Monilethrix is an autosomal dominant hair disease that results in short, fragile, broken hair that appears beaded. Mutations in the person basic hair keratins hHb1 and hHb6 have recently been reported in this disease. These changes in the helix-encoding area in the hair-specific keratins hHb1 and hHb6 may represent different novel heterozygous point mutations of the same codon in exon 7 of the hHb6 gene. A mutational hotspot may exist in the helix termination motif of hHb6. A missense mutation in the type II hair keratin hHb3 has been shown to be associated with monilethrix.
The hair may lack luster, and there may be patchy areas of hair loss (alopecia). Another common symptom may be the appearance of elevated spots (papules) surrounding the hair follicles that may be covered with gray or brown crusts or scales (perifollicular hyperkeratosis). The nodes seemed to represent normal hair growth, with hair medulla & regular number and structure of cortical cells. The internodes in contracts seemed to be more transparent and characterised by a lower number and wrinkling of cortical to be more transparent and characterised by a lower number and wrinkling of cortical cells leading to the fragility of the hair.
There is no effective treatment is available of Monilethrix. Monilethrix although improvement has been reported with oral steroids, retinoid, griseofulvin and topical minoxidil. The avoidance of trauma is the most effective method of managing this anomaly. A wig can be used. Congenitally abnormal hair tends to have an increased susceptibility to weathering and cosmetic damage (e.g., sunlight exposure, dyeing, bleaching, perming, curling), which can prevent hair from growing to its maximum length.
