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While approved in Australia as an anti\u2010rejection medication for renal transplant patients, there is mounting evidence regarding the utility of oral and topical sirolimus in treating a plethora of dermatological conditions or conditions with cutaneous manifestations. Our aim was to present an overview of the evidence for current usage and breadth of the application of sirolimus in dermatology. We carried out a systematic review of all the literature published up to 31 August 2019 on oral and topical sirolimus with respect to dermatological conditions or conditions otherwise relevant to dermatology. While 3368 papers were initially produced in our search, 238 papers met our inclusion criteria and were examined in our review. The conditions examined were categorised into genodermatoses (9 conditions), infection (1 condition), inflammatory\/autoimmune (10 conditions), neoplasm (3 conditions) and vascular (17 conditions). We extracted data on first author, publication year, journal, characteristics of the study and study patients, condition, drug modalities, drug efficacy, side effects, blood level of mTORI, co\u2010interventions and follow\u2010up. While there is level 1 evidence for the efficacy of sirolimus in conditions such as tuberous sclerosis complex (TSC) and GVHD prophylaxis, for many other conditions, the evidence is limited to level 4 evidence. Regarding oral systemic therapy, dosing regimens varied with the most common for children 0.8mg\/m<jats:sup>2<\/jats:sup> twice daily and for adults 1\u2009mg twice daily. Doses were often adjusted to reach a typical trough level of between 5 and 15\u2009ng\/mL, though targets often varied. In the overall majority of cases, side effects were minimal or tolerable, including mucositis, cytopenias, lipid abnormalities and nausea\/vomiting, and only a few cases had to stop due to adverse effects. Regarding topical therapy, concentration of formulations varied from 0.1% to 1% and were compounded into creams, ointments or gels and administered typically once or twice per day. The most common side effect was skin irritation. There were a number of limitations to our study. In particular, many of the published studies were case reports or case series with no comparator arm, leading to susceptibility of bias in conclusions drawn, in particular a high likelihood of publication bias. Given the heterogeneity amongst studies, comparisons or aggregation of results was difficult. There continues to be growing use of oral and topical sirolimus in dermatological conditions. It provides new therapeutic options to patients where previous therapies have either failed or are limited due to toxicity. However, further studies are warranted.<\/jats:p>","DOI":"10.1111\/ajd.13671","type":"journal-article","created":{"date-parts":[[2021,7,30]],"date-time":"2021-07-30T11:33:12Z","timestamp":1627644792000},"page":"461-469","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Systematic review of sirolimus in dermatological conditions"],"prefix":"10.1111","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9740-1297","authenticated-orcid":false,"given":"Andrew W","family":"Swarbrick","sequence":"first","affiliation":[{"name":"Department of Dermatology Sir Charles Gairdner Hospital Nedlands Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6768-4687","authenticated-orcid":false,"given":"Aaron J","family":"Frederiks","sequence":"additional","affiliation":[{"name":"Royal Perth Hospital Perth Australia"},{"name":"School of Medicine University of Western Australia Perth Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rachael S","family":"Foster","sequence":"additional","affiliation":[{"name":"Department of Dermatology Sir Charles Gairdner Hospital Nedlands Australia"},{"name":"Department of Dermatology Perth Children\u2019s Hospital Nedlands Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,7,30]]},"reference":[{"key":"e_1_2_9_2_1","unstructured":"Therapeutic Goods Administration.Australian Register of Therapeutic Goods.2019. 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