Abstract
Priasis rubra pilaris (PRP) is the relatively rare inflammatory skin disease of unknown etiology. As possible trigger factors are considered infections, malignances or autoimmune disturbances, which were observed to precede occurrence of the disease.
According to age, morphological features and clinical course the disease is classified into six forms. The most common is classic adult form, representing over 50% of PRP cases, which is also characterized by high spontaneous remission.
Typical skin lesion include erythematosquamous salmon-colored plaques and areas often evolving in erytroderma with well demarcated islands of normal skin. Follicular papules, palmoplantar keratoderma.
Histology shows hyperkeratosis with alternative orthokeratois and parakeratosis in a chessboard pattern and focal acantholytic dysceratosis. Mainstay of the treatment is systemic therapy with retinoids, methotrexate and cyclosporine.
Good effect of „biologics“ used in psoriasis was observed also in the therapy of PRP.