Pityriasis rubra pilaris (PRP) includes a spectrum of rare chronic inflammatory disorders with papulosquamous eruptions of unknown cause. Different etiologies have been proposed such as vitamin A metabolism dysfunction, association with autoimmune disorders, infection or malignancies. However, PRP seems to be a polygenic skin disorder. Classical systemic treatment is empirical and includes retinoids and methotrexate; however, only few series on treatments exist. Recently there has been an increasing number of reports documenting that new biologicals and in particular TNF-α blockers are safe and effective.

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