The skin changes usually start between the ages
of 7 and 10 years with follicular atrophy (so-called atrophoderma
vermiculatum), particularly on the cheeks, and with discoloration of the
lips and hands (cyanosis-like). Milia-like papules with telangiectasias
develop in adulthood, and basal cell carcinomas are a frequent complication
of this syndrome, but appear later in life than in Bazex Syndrome. Basal
cell carcinomas with milia and coarse, sparse hair syndrome or congenital
hypotrichosis with milia syndrome. Microscopically, a widespread lack of
elastin in many skin areas with clumping of elastic components in other
areas can be identified. Dermal collagen fibrils may have signs of
hyalinization and vacuolization. Skin can get the aspect of solar elastosis.
The eyebrows and eyelashes may either show an irregular, patchy distribution
with distorted and maldirected growth, or may be missing completely.