An autosomal dominant inheritance has been postulated for the juvenile form of the disease. The classic adult form has the best prognosis, with resolution usually over a 3-year period.
Generalized involvement is characterized by yellow-orange erythema with desquamation.
Pruritus is usually mild or absent. Ultraviolet B phototherapy may exacerbate the disease. High-dose vitamin A in excess of 200,000 IU daily has been used but can cause liver or central nervous system toxicity. Treatment with oral retinoids such as etretinate or acitretin is indicated in men and postmenopausal women for the treatment of pityriasis rubra pilaris. Remission of up to 6 months was sustained in some patients after the drug was withdrawn.