Erosive pustular dermatosis of the scalp (EPDS) is a chronic condition characterised by erosive plaques and subsequent scarring alopecia as a result of local trauma or inflammation. A number of therapeutic approaches have been described in the literature but there is no consensus of opinion on optimal treatment of disease.To provide evidence-based recommendations for topical and systemic treatment of adult patients with EPDS by performing a systematic review.MEDLINE, MEDLINE in-process, EMBASE and Cochrane databases were searched from inception to 26th June 2019 in accordance with PRISMA guidelines for studies involving of adult patients treated for EPDS with at least one reported response to treatment. The study was registered on PROSPERO. Texts were reviewed independently by two authors. Risk of bias and quality were assessed using the Quality Appraisal Checklist for Case Series Studies .A total of 75 studies were included, involving 171 patients. Many treatments have been reported in the literature with varying degrees of therapeutic success. Results were highly heterogeneous and methodological quality was very low. We were unable to perform a meta-analysis on the data.The limited available evidence supports use of topical corticosteroids, with or without oral zinc, followed by maintenance therapy with topical calcineurin inhibitors as effective in managing this condition. Topical photodynamic therapy is also potentially beneficial in the management of EPDS. Prospective, comparative, randomised controlled trials are required in order to provide further evidence to guide treatment.