Any race can be affected. PRP occurs equally among male and female patients. The acquired form of PRP has a bimodal age distribution with peaks in the first and fifth decades but can begin at any age. The familial form of PRP has a gradual onset, whereas the acquired form has an acute onset. The disease typically spreads in a craniocaudal direction.
Patients first notice redness and scaling of the face and scalp.
This is often followed by redness and thickening of the palms and soles. The lesions may expand and coalesce to cover the entire body. Follicular hyperkeratosis is commonly seen on the dorsal aspects of the proximal phalanges, elbows and wrists. Palmoplantar keratoderma occurs in most patients and tends to have an orange hue. Painful fissures may develop in patients with palmoplantar keratoderma.