Pityriasis Rubra Pilaris (PPR) is a rare dermatosis and its incidence, etiology and pathogenesis are uncertain. Classification systems for this dermatosis, consider its various clinical manifestations that subdivide it into 5 clinical types, based on characteristics, such as age of involvement, extension and prognosis. Soon, This article aims to present a case report of PRP type (I) that occurred in the municipality of Petrolina (Pernambuco -Brazil) in 2021, with excellent and fast response to the use of Methotrexate, as well as providing a descriptive overview of the pathology in question and its different forms of treatment through a brief review and discussion about this unusual comorbidity. To the preparation of this review, the PubMed, Medline and Lilacs in the period from March 2016 to August 2021. For collection of the articles, the descriptors, pityriasis rubra pilaris and treatment and considered only articles in the English language. 34 articles were considered for the extraction of data, which were analyzed independently in forms standardized. It was observed that promising results have been reported using of immunobiologicals. However, the therapeutic approach for cases of PRP remains challenging, there is a gap in guidelines and algorithms for treatment, as well as as efficient clinical studies to guide interventionist action, given the unsatisfactory outcome of some lines of treatment, the particularities of patients, as well as the high cost of biological therapies currently used.
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