title: Classification of Rhinoentomophthoromycosis into Atypical, Early, Intermediate, and Late Disease: A Proposal.
abstract: BACKGROUND
  Rhinoentomophthoromycosis, or rhino-facial conidiobolomycosis, is a rare, grossly disfiguring disease due to an infection with entomophthoralean fungi. We report a case of rhinoentomophthoromycosis from Gabon and suggest a staging system, which provides information on the prognosis and duration of antifungal therapy.
  
  
  METHODS
  We present a case of rhinoentomophthoromycosis including the histopathology, mycology, and course of disease. For the suggested staging system, all cases on confirmed rhinoentomophthoromycosis published in the literature without language restriction were eligible. Exclusion criteria were missing data on (i) duration of disease before correct diagnosis, (ii) outcome, and (iii) confirmation of entomophthoralean fungus infection by histopathology and/or mycology. We classified cases into atypical (orbital cellulitis, severe pain, fever, dissemination), early, intermediate, and late disease based on the duration of symptoms before diagnosis. The outcome was evaluated for each stage of disease.
  
  
  RESULTS
  The literature search of the Medpilot database was conducted on January 13, 2014, (updated on January 18, 2015). The search yielded 8,333 results including 198 cases from 117 papers; of these, 145 met our inclusion criteria and were included in the final analysis. Median duration of treatment was 4, 3, 4, and 5 months in atypical, early, intermediate, and late disease, respectively. Cure rates were clearly associated with stage of disease and were 57%, 100%, 82%, and 43% in atypical, early, intermediate, and late disease, respectively.
  
  
  CONCLUSIONS
  We suggest a clinical staging system that underlines the benefit of early case detection and may guide the duration of antifungal treatment. The scientific value of this classification is its capacity to structure and harmonize the clinical and research approach towards rhinoentomophthoromycosis.
authors.affiliation: Institut für Tropenmedizin, Eberhard Karls Universität Tübingen and Deutsches Zentrum für Infektionsforschung, Tübingen, Germany; Centre de Recherches Médicales de Lambaréné (CERMEL), Albert Schweitzer Hospital Lambaréné, Lambaréné, Gabon; Centre de Recherche Médicale de la Ngounié, Fougamou, Gabon.
authors.forename: Christian G
authors.initials: CG
authors.lastname: Blumentrath
authors.affiliation: Institut für Tropenmedizin, Eberhard Karls Universität Tübingen and Deutsches Zentrum für Infektionsforschung, Tübingen, Germany; Centre de Recherches Médicales de Lambaréné (CERMEL), Albert Schweitzer Hospital Lambaréné, Lambaréné, Gabon; Center of Tropical Medicine and Travel Medicine, Department of Infectious Diseases, Division of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Martin P
authors.initials: MP
authors.lastname: Grobusch
authors.affiliation: Centre de Recherche Médicale de la Ngounié, Fougamou, Gabon.
authors.forename: Pierre-Blaise
authors.initials: PB
authors.lastname: Matsiégui
authors.affiliation: Evidat, Statistical Apps and Consulting, Sereetz, Germany.
authors.forename: Friedrich
authors.initials: F
authors.lastname: Pahlke
authors.affiliation: Institut für Tropenmedizin, Eberhard Karls Universität Tübingen and Deutsches Zentrum für Infektionsforschung, Tübingen, Germany; Centre de Recherches Médicales de Lambaréné (CERMEL), Albert Schweitzer Hospital Lambaréné, Lambaréné, Gabon; Centre de Recherche Médicale de la Ngounié, Fougamou, Gabon.
authors.forename: Rella
authors.initials: R
authors.lastname: Zoleko-Manego
authors.affiliation: Département de Parasitologie, Mycologie et Médicine Tropicale, Université des Sciences de la Santé, Libreville, Gabon.
authors.forename: Solange
authors.initials: S
authors.lastname: Nzenze-Aféne
authors.affiliation: Département d'Anatomie Pathologique et d`Histologie et Embryologie, Université des Sciences de la Santé, Libreville, Gabon.
authors.forename: Barthélemy
authors.initials: B
authors.lastname: Mabicka
authors.affiliation: Institute of Microbiology, Università Cattolica del Sacro Cuore, Rome, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Sanguinetti
authors.affiliation: Institut für Tropenmedizin, Eberhard Karls Universität Tübingen and Deutsches Zentrum für Infektionsforschung, Tübingen, Germany; Centre de Recherches Médicales de Lambaréné (CERMEL), Albert Schweitzer Hospital Lambaréné, Lambaréné, Gabon.
authors.forename: Peter G
authors.initials: PG
authors.lastname: Kremsner
authors.affiliation: Centre de Recherches Médicales de Lambaréné (CERMEL), Albert Schweitzer Hospital Lambaréné, Lambaréné, Gabon; Institute of Medical Microbiology, University Hospital Münster, Münster, Germany.
authors.forename: Frieder
authors.initials: F
authors.lastname: Schaumburg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D020091:Entomophthorales / Q000302:isolation & purification*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012220:Rhinitis / Q000145:classification*; D020096:Zygomycosis / Q000145:classification* / Q000175:diagnosis / Q000188:drug therapy