The scalp is often dry and scaly. Scalp alopecia begins sometime in the first two decades of life and becomes a major complaint by the third or fourth decade. The face and especially the cheeks are often erythematous. The scalp can have multiple follicular pustules which are most prominent in the occipital and nuchal areas. Follicular keratotic papules are often located on the trunk and extensor areas of the limbs. Histology of scalp skin biopsies show epidermal hyperplasia and an extensive perifollicular inflammatory infiltrate.
Enamel hypoplasia result in multiple and recurrent caries and loss of teeth. The nails are often dystrophic.
