Conidiobolomycosis

Conidiobolomycosis
Other names: Rhinoentomophthoromycosis[1]
Conidiobolomycosis: Reddish hard swelling over the lower half of face with thickening of the lower lip
SpecialtyInfectious disease[2]
SymptomsFirm painless swelling in nose, sinuses, cheeks and upper lips, blocked nose, runny nose, nose bleed[3]
Complications
Usual onsetSlowly progressive[5]
DurationLong term[3]
CausesConidiobolus coronatus.[3]
Risk factorsTropical forests of South and Central America, West Africa, Southeast Asia[4]
Diagnostic methodMedical imaging, biopsy, microscopy, culture, histopathology[4]
Differential diagnosisSoft tissue tumors[3]
TreatmentAntifungals, surgical debridement[5]
MedicationItraconazole, Potassium iodide[5] Severe disease: Amphotericin B[4]
PrognosisLongterm morbidity: facial disfigurement,[3] good response to treatment[6]
FrequencyRare, M>F[3] adults>children[4]
DeathsRare[5]

Conidiobolomycosis is a long term fungal infection that is typically found just under the skin of the nose, sinuses, cheeks and upper lips.[2][3] It may present with a nose bleed or a blocked or runny nose.[3] Typically there is a firm painless swelling which can slowly extend to the nasal bridge and eyes, sometimes causing facial disfigurement.[5]

Most cases are caused by Conidiobolus coronatus, a fungus found in soil and in the environment in general, which can infect healthy people.[3] It is usually acquired by breathing in the spores of the fungus, but can be by direct infection through a cut in the skin such as an insect bite.[2][3]

The extent of disease may be seen using medical imaging such as CT scanning of the nose and sinus.[3] Diagnosis may be confirmed by biopsy, microscopy, culture and histopathology.[3][4] Treatment is with long courses of antifungals and sometimes cutting out infected tissue.[5] Generally, treatment is with triazoles, preferably itraconazole.[4] A second choice is potassium iodide, either alone or combined with itraconazole.[4] In severe widespread disease, amphotericin B may be an option.[4] The condition has a good response to antifungal treatment,[6] but can recur.[7] The infection is rarely fatal.[5]

The condition is rare, but occurs more frequently in adult males working or living in the tropical forests of South and Central America, West Africa and Southeast Asia.[3][4] The first case in a human was described in Jamaica in 1965.[3]

Signs and symptoms

The infection presents with firm lumps just under the skin of the nose, sinuses, upper lips and cheeks.[3] The swelling is painless and may feel "woody".[7] Sinus pain may occur.[5] Infection may extend to involve the nasal bridge, face and eyes, sometimes resulting in facial disfigurement.[3] The nose may feel blocked or have a discharge, and may bleed.[3]

Cause

Conidiobolus coronatus[8]

Conidiobolomycosis is a type of Entomophthoromycosis, the other being basidiobolomycosis, and is caused by mainly Conidiobolus coronatus, but also Conidiobolus incongruus and Conidiobolus lamprauges,[3] fungi belonging to the order Entomophthorales.[4]

Mechanism

Conidiobolomycosis chiefly affects the central face, usually beginning in the nose before extending onto paranasal sinuses, cheeks, upper lip and pharynx.[4] The disease is acquired usually by breathing in the spores of the fungus, which then infect the tissue of the nose and paranasal sinuses, from where it slowly spreads.[3] It can attach to underlying tissues, but not bone.[3][4] It can be acquired by direct infection through a small cut in the skin such as an insect bite.[2] Thrombosis, infarction of tissue and spread into blood vessels does not occur.[3] Deep and systemic infection is possible in people with a weakened immune system.[3] Infection causes a local chronic granulomatous reaction.[5]

Diagnosis

The condition is typically diagnosed after noticing facial changes.[5] The extent of disease may be seen using medical imaging such as CT scanning of the nose and sinus.[3] Diagnosis can be confirmed by biopsy, microscopy, and culture.[3] Histology reveals wide but thin-walled fungal filaments with branching at right-angles.[4] There are only a few septae.[4] The fungus is fragile and hence rarely isolated.[1] An immunoallergic reaction might be observed, where a local antigen–antibody reaction causes eosinophils and hyaline material to surround the organism.[4] Molecular methods may also be used to identify the fungus.[4]

Differential diagnosis

Differential diagnosis includes soft tissue tumors.[3] Other conditions that may appear similar include mucormycosis, cellulitis, rhinoscleroma and lymphoma.[5]

Treatment and outcome

Before, during and after treatment. (A) Healthy young adult. (B) Before treatment (month 0). (C) After treatment with fluconazole and terbinafine (month 18). (D) After 14 months without therapy (month 32).[9]

Treatment is with long courses of antifungals and sometimes cutting out infected tissue.[5] Generally, treatment is with triazoles, preferably itraconazole.[4] A second choice is potassium iodide, either alone or combined with itraconazole.[4] In severe widespread disease, amphotericin B may be an option.[4] The condition has a good response to antifungal treatment,[6] but can recur.[7] The infection is rarely fatal.[5]

Epidemiology

The disease is rare, occurring mainly in those working or living in the tropical forests of West Africa, Southeast Asia, South and Central America,[3] as well India, Saudi Arabia and Oman.[4] Conidiobolus species have been found in areas of high humidity such as the coasts of the United Kingdom, eastern United States and West Africa.[5]

Adults are affected more than children.[4]

History

The condition was first reported in 1961 in horses in Texas.[3] The first case in a human was described in 1965 in Jamaica.[3] Previously it was thought to only infect insects.[3]

Other animals

Conidiobolomycosis affects spiders, termites and other arthropods.[3] The condition has been described in dogs, horses, sheep and other mammals.[10] Affected horses typically present with irregular lumps in one or both nostrils that cause obstruction, bloody nasal discharge and noisy abnormal breathing.[10]

References

  1. 1 2 Arora, Pooja; Sardana, Kabir; Madan, Anjali; Khurana, Nita (2016). "An Old Woman with a Lump". Indian Journal of Dermatology. 61 (6): 697–699. doi:10.4103/0019-5154.193705. ISSN 0019-5154. PMC 5122299. PMID 27904202. Archived from the original on 2021-08-29. Retrieved 2021-08-26.
  2. 1 2 3 4 "ICD-11 - ICD-11 for Mortality and Morbidity Statistics". icd.who.int. Archived from the original on 1 August 2018. Retrieved 5 June 2021.
  3. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 Chander, Jagdish (2018). Textbook of Medical Mycology (4th ed.). New Delhi: Jaypee Brothers Medical Publishers Ltd. pp. 599–603. ISBN 978-93-86261-83-0. Archived from the original on 2021-08-28. Retrieved 2021-06-06.
  4. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 Queiroz-Telles, Flavio; Fahal, Ahmed Hassan; Falci, Diego R.; Caceres, Diego H.; Chiller, Tom; Pasqualotto, Alessandro C. (November 2017). "Neglected endemic mycoses". The Lancet. Infectious Diseases. 17 (11): e367–e377. doi:10.1016/S1473-3099(17)30306-7. ISSN 1474-4457. PMID 28774696. Archived from the original on 2021-08-27. Retrieved 2021-08-25.
  5. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 Sherchan, Robin; Zahra, Farah (2021). "Entomophthoromycosis". StatPearls. StatPearls Publishing.
  6. 1 2 3 Gupta, Nitin; Sonej, Manish (March 2019). "JCDR - Conidiobolus coronatus, Conidiobolus incongruus, Entomophthoramycosis". Journal of Clinical and Diagnostic Research. 13 (3). doi:10.7860/JCDR/2019/40142.12701. Archived from the original on 2021-08-29. Retrieved 2021-08-29.
  7. 1 2 3 Das, Sudip Kumar; Das, Chiranjib; Maity, Amit Bikram; Maiti, Prasanta Kumar; Hazra, Tapan Kanti; Bandyopadhyay, Saumendra Nath (November 2019). "Conidiobolomycosis: An Unusual Fungal Disease—Our Experience". Indian Journal of Otolaryngology and Head & Neck Surgery. 71 (Suppl 3): 1821–1826. doi:10.1007/s12070-017-1182-6. ISSN 2231-3796. PMID 31763253. Archived from the original on 2021-08-28. Retrieved 2021-06-05.
  8. ↑ Nie, Yong; Yu, De-Shui; Wang, Cheng-Fang; Liu, Xiao-Yong; Huang, Bo (24 August 2021). "A taxonomic revision of the genus Conidiobolus (Ancylistaceae, Entomophthorales): four clades including three new genera". MycoKeys. pp. 55–81. doi:10.3897/mycokeys.66.46575. Archived from the original on 24 August 2021. Retrieved 6 June 2021.
  9. ↑ Blumentrath, Christian G.; Grobusch, Martin P.; Matsiégui, Pierre-Blaise; Pahlke, Friedrich; Zoleko-Manego, Rella; Nzenze-Aféne, Solange; Mabicka, Barthélemy; Sanguinetti, Maurizio; Kremsner, Peter G.; Schaumburg, Frieder (1 October 2015). "Classification of Rhinoentomophthoromycosis into Atypical, Early, Intermediate, and Late Disease: A Proposal". PLoS Neglected Tropical Diseases. 9 (10). doi:10.1371/journal.pntd.0003984. ISSN 1935-2727. PMID 26426120. Archived from the original on 23 September 2021. Retrieved 13 September 2021.
  10. 1 2 Sellon, Debra C.; Long, Maureen T. (2007). Equine Infectious Diseases. St. Louis, Missouri: Saunders Elsevier. p. 417. ISBN 978-1-4160-2406-4. Archived from the original on 2021-08-28. Retrieved 2021-06-06.
Classification
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