Conidiobolomycosis B48.7

Last updated on: 29.10.2020

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Synonym(s)

Conidiobolomycosis

Pathogen
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Entomophthorales (fly killer fungi). Entomophthorales are an order within the fungi, which used to belong to the zygomycetes, but now forms a separate group. They are preferably parasites that attack insects (Entomophthorales - Greek: Entomo= insect - phthor = destruction) but can also appear as opportunistic systemic mycosis. The most frequent pathogen of the Conidiobolomycosis is the species Conodiobolus coronatus.

Occurrence/Epidemiology
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Most cases were reported from Equatorial Africa (50%), India (30%), Central America (15%). Rarely Asia (2%) and Europe (1%).

Manifestation
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30-40 years, m:w=1:3. >90% of the patients were otherwise healthy. 8% of the patients had systemic diseases (immunodeficiencies).

Clinical features
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Deeply cutaneously localized but also systemically occurring mycosis, which is spread worldwide, preferably in tropical regions (in the past also called rhinophycomycosis or rhinoentomophoramycosis). Infection occurs via inhalation or via small injuries to the skin or mucous membranes.

In a larger overview, most cases (80%) presented with long-term (average 13 months), gradually progressive, less painful, shapeless swellings of the centrofacial facial areas (nose, lips, glabella, eyelids) with infestation of skin, skin-near mucous membranes as well as skin-near osseous structures (facial elephantiasis). Systemic involvement (trachea, thyroid gland, mediastinum, lung, spleen, kidney, etc.) was observed in about 10% of cases.

Laboratory
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Non-specific

Diagnosis
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Histology with detection of the pathogens; detection of the Splendore-Hoeppli phenomenon. Culture.

Differential diagnosis
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  • Melkersson-Rosenthal syndrome (analogous pattern, but lacks the bulky swelling of the central part of the face)
  • Basidiobolomycosis: Related tropical mycosis that mainly affects the trunk and extremities.
  • Chronic lymphedema after bacterial infection (e.g. chronic erysipelas)

Therapy
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antifungal (azole antifungals such as ketoconazole, itraconazole, fluconazole, or amphotericin B)

Literature
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  1. Bachelet JT et al (2014) Conidiobolus coronatus infections revealed by a facial tumor. Rev Stomatol Chir Maxillofac Chir Orale 115:114-117
  2. Bento DP et al (2010) Atypical presentation of entomophthoromycosis caused by Conidiobolus coronatus. Med Mycol 48:1099-1104
  3. Choon SE et al (2012) Conidiobolomycosis in a young Malaysian woman showing chronic localized fibrosing leukocytoclastic vasculitis: a case report and meta-analysis focusing on clinicopathologic and therapeutic correlations with outcome. At J Dermatopathol 34:511-522
  4. Chowdhary A et al (2010) Rhinoentomophthoromycosis due to Conidiobolus coronatus. A case report and an overview of the disease in India. Med Mycol 48: 870-879
  5. Isa-Isa R et al (2012) Rhinofacial conidiobolomycosis (entomophthoramycosis). Clin Dermatol 30: 409-412
  6. Leopairut J et al (2010) Rhinofacial entomophthoramycosis; a case series and review of the literature. Southeast Asian J Trop Med Public Health 41:928-935

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Last updated on: 29.10.2020