Limits...
Classification of Rhinoentomophthoromycosis into Atypical, Early, Intermediate, and Late Disease: A Proposal.

Blumentrath CG, Grobusch MP, Matsiégui PB, Pahlke F, Zoleko-Manego R, Nzenze-Aféne S, Mabicka B, Sanguinetti M, Kremsner PG, Schaumburg F - PLoS Negl Trop Dis (2015)

Bottom Line: 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.Cure rates were clearly associated with stage of disease and were 57%, 100%, 82%, and 43% in atypical, early, intermediate, and late disease, respectively.We suggest a clinical staging system that underlines the benefit of early case detection and may guide the duration of antifungal treatment.

View Article: PubMed Central - PubMed

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.

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.

Findings: 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus

Histopathology.Histopathological picture of hyphae of Entomophthorales after KOH-staining (A), after hematoxylin eosin stain (HE-stain) (B and C), and after Grocott-Gomori's methenamine silver stain (GMS-stain) (D). The pauci-septated, right-angled branching fungal hyphae have an irregular diameter of 5–12 μm with drumstick-like distended ends (A). The “Splendore-Hoeppli phenomenon” is characterized by a peri-hyphal amorphous eosinophilic material (B). Fungal hyphae are visible in transversal (C) or longitudinal cuts (D).
© Copyright Policy
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4591341&req=5

pntd.0003984.g003: Histopathology.Histopathological picture of hyphae of Entomophthorales after KOH-staining (A), after hematoxylin eosin stain (HE-stain) (B and C), and after Grocott-Gomori's methenamine silver stain (GMS-stain) (D). The pauci-septated, right-angled branching fungal hyphae have an irregular diameter of 5–12 μm with drumstick-like distended ends (A). The “Splendore-Hoeppli phenomenon” is characterized by a peri-hyphal amorphous eosinophilic material (B). Fungal hyphae are visible in transversal (C) or longitudinal cuts (D).

Mentions: Correct diagnosis of entomophthoromycoses is a challenge, as biopsies rarely reveal characteristic fungal hyphae in potassium hydroxide smears (Fig 3A). Hematoxylin eosin staining demonstrates an intense Splendore-Hoeppli-Phenomenon around unstained fungal hyphae and massive tissue infiltration by eosinophils; Gomori methamine silver and periodic acid Schiff coloration stain fungal cell walls (Fig 3B, 3C and 3D) [2,12,14,15]. Mycological cultures become positive in approximately 50% of cases [2,12,14,15]. Flat, waxy, white colonies grow within 3–10 days on Sabouraud agar (37°C), which become brownish over time [14]. Crown shaped, villous conidia are characteristic of C. coronatus [14,16,17]. Serologic, intracutaneous, and PCR tests are available in specialised laboratories [12,18].


Classification of Rhinoentomophthoromycosis into Atypical, Early, Intermediate, and Late Disease: A Proposal.

Blumentrath CG, Grobusch MP, Matsiégui PB, Pahlke F, Zoleko-Manego R, Nzenze-Aféne S, Mabicka B, Sanguinetti M, Kremsner PG, Schaumburg F - PLoS Negl Trop Dis (2015)

Histopathology.Histopathological picture of hyphae of Entomophthorales after KOH-staining (A), after hematoxylin eosin stain (HE-stain) (B and C), and after Grocott-Gomori's methenamine silver stain (GMS-stain) (D). The pauci-septated, right-angled branching fungal hyphae have an irregular diameter of 5–12 μm with drumstick-like distended ends (A). The “Splendore-Hoeppli phenomenon” is characterized by a peri-hyphal amorphous eosinophilic material (B). Fungal hyphae are visible in transversal (C) or longitudinal cuts (D).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4591341&req=5

pntd.0003984.g003: Histopathology.Histopathological picture of hyphae of Entomophthorales after KOH-staining (A), after hematoxylin eosin stain (HE-stain) (B and C), and after Grocott-Gomori's methenamine silver stain (GMS-stain) (D). The pauci-septated, right-angled branching fungal hyphae have an irregular diameter of 5–12 μm with drumstick-like distended ends (A). The “Splendore-Hoeppli phenomenon” is characterized by a peri-hyphal amorphous eosinophilic material (B). Fungal hyphae are visible in transversal (C) or longitudinal cuts (D).
Mentions: Correct diagnosis of entomophthoromycoses is a challenge, as biopsies rarely reveal characteristic fungal hyphae in potassium hydroxide smears (Fig 3A). Hematoxylin eosin staining demonstrates an intense Splendore-Hoeppli-Phenomenon around unstained fungal hyphae and massive tissue infiltration by eosinophils; Gomori methamine silver and periodic acid Schiff coloration stain fungal cell walls (Fig 3B, 3C and 3D) [2,12,14,15]. Mycological cultures become positive in approximately 50% of cases [2,12,14,15]. Flat, waxy, white colonies grow within 3–10 days on Sabouraud agar (37°C), which become brownish over time [14]. Crown shaped, villous conidia are characteristic of C. coronatus [14,16,17]. Serologic, intracutaneous, and PCR tests are available in specialised laboratories [12,18].

Bottom Line: 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.Cure rates were clearly associated with stage of disease and were 57%, 100%, 82%, and 43% in atypical, early, intermediate, and late disease, respectively.We suggest a clinical staging system that underlines the benefit of early case detection and may guide the duration of antifungal treatment.

View Article: PubMed Central - PubMed

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.

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.

Findings: 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus