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

Abstract

BackgroundRhinoentomophthoromycosis, 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.MethodsWe 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.FindingsThe 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.ConclusionWe 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.

Human Pathogenic Entomophthorales

“…The disease occurs more frequently in males than in females. In some areas of Africa, the male-to-female ratio is close to 3:1, whereas all cases of the world together showed a ratio of 8:1, very close to data from previous reports (129,130). This trend of high infection rates in males may be related to occupational activities.…”

“…In addition, Choon et al (171) suggested that chronic lymphedema could also play a role in the facial deformity observed in extreme chronic cases of rhinoconidiobolomycosis (118,130). According to the CLFLCV hypothesis, long-standing lymphostasis could induce permanent tissue overgrowth, as in elephantiasis cases (130). Choon et al (171) indicated that in chronic cases, the immune response does not control fungal growth.…”

“…It is usually found bilaterally, but some unilateral cases have also been mentioned (169). If not treated, the infection becomes chronic (several months to even years in some extreme cases) and progresses to indolent facial deformity (130). In early and chronic conidiobolomycosis cases, the terms atypical, early, intermediate, and late disease have been suggested (130).…”

Entomophthoramycosis: a neglected tropical mycosis

“…Atypical mucocutaneous conidiobolomycosis, defined by Blumentrath et al [45] as either facial infection in a region other than the nose and maxillary sinus or infection of a nonfacial cutaneous site has also been reported.…”

A Rhinofacial Conidiobolus coronatus Fungal Infection Presenting as an Intranasal Tumour

“…1,3,15,16 Blumentrath et al proposed a treatment strategy according to stages of disease progression; based on these classifications, the current case would be considered intermediate due to the incubation period and vestibular skin involvement and a surgical approach would not be recommended. 17 However, the current patient had non-responsive bleeding and a blocked airway; as such, his quality of life immediately improved following surgical debridement.…”