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Premature loss of primary teeth may present a diagnostic dilemma to paediatric dentists.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>To identify systemic conditions associated with PLPT and develop a clinical aid.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>OVID Medline, Embase and Web of Science were searched up to March 2023. Citation searching of review publications occurred. Exclusion occurred for conference abstracts, absence of PLPT and absence of English\u2010language full text.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seven hundred and ninety\u2010one publications were identified via databases and 476 by citation searching of review articles. Removal of 390 duplicates occurred. Following the exclusion of 466 records on abstract review, 411 publications were sought for retrieval, of which 142 met inclusion criteria. Thirty\u2010one systemic conditions were identified. For 19 conditions, only one publication was identified. The majority of publications, 91% (<jats:italic>n<\/jats:italic>\u2009=\u2009129), were case reports or series. Most publications, 44% (<jats:italic>n<\/jats:italic>\u2009=\u200962), were related to hypophosphatasia, and 25% (<jats:italic>n<\/jats:italic>\u2009=\u200935) were related to Papillon\u2013Lef\u00e8vre. Diagnostic features were synthesised, and a clinical aid was produced by an iterative consensus approach.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>A diverse range of systemic diseases are associated with PLPT. Evidence quality, however, is low, with most diseases having a low number of supporting cases. 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