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Based on FUMHD cases identified in a MEDLINE literature search, demographic, treatment, and outcome data were analyzed. An FUMHD mortality risk score is proposed based on the likelihood ratios of risk factors for a fatal outcome. Our FUMHD case had marked leukopenia and thrombocytopenia at admission. He recovered without systemic immunosuppressive treatment. Literature review revealed 119 FUMHD cases. Overall lethality was 14\/119 (12%, CI 6\u201317%), and lethality in children was lower (1\/54, 2%, CI 0\u20136%) compared to adults (13\/65, 20%, CI 11\u201331%). Risk factors for a fatal outcome (likelihood ratio;<jats:italic>P<\/jats:italic>) were sepsis (24.97,<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001), adult vs. pediatric patient age (11.19;<jats:italic>P<\/jats:italic>\u2009=\u20090.001), systemic involvement (19.97,<jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001), and mucosal involvement (4.58;<jats:italic>P<\/jats:italic>\u2009=\u20090.032). The proposed FUMHD mortality risk score\u2009=\u2009Age\/10\u2009+\u20094\u2009+\u20094 (if systemic involvement)\u2009+\u20091 (if mucosal involvement) was discriminative (sensitivity 93%, specificity 77%). In FUMHD, immune\u2010suppressive treatment intensity should be balanced against the mortality risk, as infectious complications are a frequent cause of death.<\/jats:p>","DOI":"10.1111\/ijd.15770","type":"journal-article","created":{"date-parts":[[2021,7,20]],"date-time":"2021-07-20T11:00:14Z","timestamp":1626778814000},"page":"401-409","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Mucha\u2010Habermann disease: a pediatric case report and proposal of a risk score"],"prefix":"10.1111","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3873-0634","authenticated-orcid":false,"given":"Martin Ernst","family":"Blohm","sequence":"first","affiliation":[{"name":"Department of Paediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"},{"name":"Division of Neonatology and Paediatric Intensive Care University Medical Center Hamburg\u2010Eppendorf Hamburg 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