Abstract
Monilethrix is a rare genetic disorder of hair characterized by beaded appearance of the hair shaft leading to hair fragility and patchy dystrophic alopecia. In this disorder, the hair shaft has alternate widenings (nodes) and constrictions (internodes) that lead to fracture of hair shaft and varying degree of alopecia. We report an eight year old Kashmiri boy who presented with diffuse hair loss since infancy. As monilethrix is a rare disease entity which prompted us to report this case.
Highlights
The word Monilethrix is derived from Latin ‘monile’ meaning ‘necklace’ and the Greek ‘thrix’ meaing ‘hair’
It’s a structural hair shaft disorder characterized by a beaded appearance due to the presence of elliptical or fusiform nodes which have a diameter of normal hair
These nodes are medullated and separated by areas of constrictions called as internodes which lack medulla. These unmedullated internodes are the sites of hair breakage leading to dystrophic alopecia [1]
Summary
The word Monilethrix is derived from Latin ‘monile’ meaning ‘necklace’ and the Greek ‘thrix’ meaing ‘hair’. It’s a structural hair shaft disorder characterized by a beaded appearance due to the presence of elliptical or fusiform nodes which have a diameter of normal hair. These nodes are medullated and separated by areas of constrictions called as internodes which lack medulla. These unmedullated internodes are the sites of hair breakage leading to dystrophic alopecia [1]. The first case of monilethrix was described by Walter Smith in 1879. Monilethrix is transmitted as an autosomal dominant trait with high penetrance but variable expressivity. There are sporadic cases of autosomal recessive inheritance reported for this disease [3]
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