Pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on the scalp and spreads caudally within a few weeks. It often results in a generalized erythroderma with sharply demarcated islands of sparing ("nappes claires"). A 65-year-old patient with severe PRP showed good clinical improvement after 8 months of treatment when treated with acitretin in combination with phototherapy and systemic glucocorticosteroids.