Citation: Colomba, C.; Scalisi, M.; Ciacio, V.; Albano, C.; Bagarello, S.; Billone, S.; Guida, M.; Giordano, S.; Canduscio, L.A.; Milazzo, M.; et al. Shanghai Fever: Not Only an Asian Disease. Pathogens 2022, 11, 1306. https://doi.org/10.3390/ pathogens11111306 Academic Editor: Philippe Lehours Received: 25 August 2022 Accepted: 27 October 2022 Published: 7 November 2022 Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affil- iations. Copyright: © 2022 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/). pathogens Systematic Review Shanghai Fever: Not Only an Asian Disease Claudia Colomba 1,2 , Michela Scalisi 1, * , Valeria Ciacio 2 , Chiara Albano 2 , Sara Bagarello 2 , Sebastiano Billone 2 , Marco Guida 2 , Salvatore Giordano 1 , Laura A. Canduscio 1 , Mario Milazzo 3 , Salvatore Amoroso 3 and Antonio Cascio 2,4 1 Division of Pediatric Infectious Diseases, “G. Di Cristina” Hospital, ARNAS Civico Di Cristina Benfratelli, 90100 Palermo, Italy 2 Department of Health Promotion, Maternal and Infant Care, Internal Medicine and Medical Specialties, University of Palermo, 90100 Palermo, Italy 3 Pediatric Surgery Unit, “G. Di Cristina” Hospital, ARNAS Civico Di Cristina Benfratelli, 90100 Palermo, Italy 4 Infectious and Tropical Diseases Unit, AOU Policlinico “P. Giaccone”, 90100 Palermo, Italy * Correspondence: michela.scalisi89@gmail.com Abstract: Objectives: To describe a case of Shanghai fever disease and to analyze other published reports in non-Asiatic countries, defining clinical characteristics and highlighting that this is not only an Asian disease. Study design: A computerized search without language restriction was conducted using PubMed and Scopus; all references listed were hand-searched to identify any other relevant literature. An article was considered eligible for inclusion in the systematic review if it reported cases with Shanghai fever described in non-Asiatic countries. Our case was also included in the analysis. Results: Ten articles reporting 10 cases of Shanghai fever disease were considered. Fever, diarrhea and ecthyma gangrenosum were the most frequent symptoms observed. Blood was the most common site of isolation for Pseudomonas aeruginosa. Three patients underwent surgery due to necrotizing enteritis and intestinal perforation. Meningitis was documented in one case. None of the patients received antipseudomonal antibiotics within 24 h of admission. The outcome was good in nine cases; only one patient died due to multiple organ failure from Pseudomonas sepsis. No common primary immune deficiency was identified in these patients. Extremely young age (<1 year) was the only host factor predisposing to Shanghai fever. Conclusions: It is important to shed light on this disease in non-Asiatic countries and take into account that it can also affect healthy children. Pediatricians, therefore, should consider Shanghai fever among diagnoses in children with community-onset diarrhea, fever and skin lesions suggestive of ecthyma gangrenosum to start an appropriate treatment sooner and to reduce the mortality in these children. Keywords: Shanghai fever; Pseudomonas aeruginosa; ecthyma gangrenosum; necrotizing enteritis; sepsis 1. Introduction Shanghai fever is a rare sepsis-associated enteric disease caused by P. aeruginosa, described mainly in previously immunologically healthy children. To our knowledge, only one case has been described in the literature in an adult patient with neutropenia. The disease was reported as early as 1918 and has been more recently defined by Chuang et al. with three criteria: (1) community-onset diarrhea with fever, (2) sepsis and (3) growth of P. aeruginosa from blood or another sterile body site. Necrotizing enteritis is the major complication of Shanghai fever and mortality reported worldwide is high [1,2]. To date, most of the reported cases regarding Shanghai fever are restricted to East Asian countries and only very few cases are described to be from North America and Europe. We report a case of Shanghai fever in a previously healthy Italian 7-month-old boy and analyze the medical literature to report the epidemiologic and clinical characteristics of cases of Shanghai fever from non-Asiatic countries. Pathogens 2022, 11, 1306. https://doi.org/10.3390/pathogens11111306 https://www.mdpi.com/journal/pathogens