ABSTRACT
Figure 10.1 ● During early infancy the scalp hairs become more coarse, wiry and twisted. Eyebrows,
eyelashes and body hair are sparse to absent (Figure 10.1) ● At puberty scalp hair is progressively lost, mainly from the vertex and scalp margins ● During adolescence a scarring alopecia of varying extent develops in a pattern
suggestive of androgenetic alopecia (Figure 10.2). Anomalies remain confined to the hair. Teeth, nails, physical and mental development are normal
● Juvenile macular degeneration
Laboratory findings
● Individual hair shafts are deeply pigmented, increased in diameter and twisted. The combination of longitudinal growing and twisting of the shaft at irregular intervals is unique
● There is a reduction in the number of follicles per unit area, with little fibrosis
Genetics and pathogenesis
● Autosomal dominant disorder ● Pathogenesis is unknown
Differential diagnosis
● Ectodermal dysplasias ● Menkes’ syndrome ● Hypotrichosis simplex of the scalp
Follow-up
The condition becomes stable and is persistent. It is more evident in males.
