Abstract: Pityriasis rubra pilaris is a rare erythematous squamous disorder. Genetics, abnormality of biochemical metabolism and endocrine, immune reaction, malignancy, drug and infection may be involved in the pathogenesis of the disease. The treatment included oral retinoids, topical corticosteroids and emollients，and TNF-α antagonists. The etiology, mechanism and treatment were reviewed in this paper.

Key words: pityriasis rubra pilaris, TNF-α antagonist