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This is a PatientPlus article. PatientPlus articles are written for doctors and so the language can be technical, however some people find that they add depth to the patient information leaflets. You may find the abbreviations record helpful.

Synonyms include zygomycosis and phycomycosis

Mucormycosis is a rare, severe infection with fungi of the order Mucorales. Rhizopus are the commonest species, with Rhizomucor, Cunninghamella, Saksenaea, and Apophysomyces species occurring less often.

These fungi are common environmental organisms that only cause disease when immunity is impaired. The spores grow, fungal hyphae invade blood vessels and produce tissue infarction. The result can be invasive, life-threatening disease. Severe infection of the facial sinuses may extend into the brain. Less common manifestations are pulmonary, cutaneous and gastrointestinal infections.

Epidemiology

The condition is very rare but often unrecognised. Invasive fungal infections have always been a problem in the immunocompromised, typically aspergillosis and candidiasis, but mucormycosis has also increased in incidence over the last decade.1

Risk factors

Impaired immunity from various causes:

Presentation

History

  • There is usually a fulminant course with considerable tissue necrosis.
  • Pyrexia is usual.
  • In rhinocerebral disease there is unilateral, retro-orbital headache and nasal stuffiness progressing to a black discharge.
  • Late symptoms from invasion of the orbital nerves and vessels include diplopia and visual field loss.
  • These are late symptoms with a poor prognosis and usually are followed by reduced consciousness.
  • Pulmonary mucormycosis presents nonspecifically with fever, dyspneoa, and cough.
  • Cutaneous mucormycosis produces cellulitis that progresses to dermal necrosis and black eschar formation.
  • Gastrointestinal mucormycosis occurs with severe malnutrition and may occur throughout the GI tract. Presentation is nonspecific, with abdominal pain, abdominal distension, nausea, and vomiting.

Examination

Only the signs of rhinocerebral mucormycosis are characteristic:

  • Cellulitis of the orbit and face progresses with discharge of black pus from the palate and nose.
  • Retro-orbital extension produces proptosis, chemosis, ophthalmoplegias and blindness.
  • As the brain is involved the level of consciousness declines.

Pulmonary and gastrointestinal symptoms are non-specific but black, necrotic lesions of the skin indicate cutaneous disease.

Investigations
  • FBC
  • Renal function (high doses of amphotericin B will be required and it is nephrotoxic)
  • Diabetic control
  • Ferritin and studies of iron status
  • Imaging - both CT and MRI are useful to assess the skull, sinuses and brain (in general, CT is better for viewing bone and MRI gives better pictures of brain and soft tissues)
  • CXR and chest CT (if chest disease is suspected)
  • Abdominal CT (may show a mass by the gut)
  • Tissue biopsy and microbiology (hyphae must be demonstrated to start treatment swiftly, culture is also required to determine the species but will take longer)
Management

Drug

  • Prompt administration of amphotericin B at high dose is essential. An adequate course lasts 4 to 6 weeks.
  • Rectify any poor control of diabetes.
  • Stop chemotherapy if it is causing neutropenia.
  • Deferoximine should also be stopped.

Surgical

  • Surgical debridement of any necrotic tissue is mandatory.
  • In rhinocerebral disease, surgery includes drainage of the sinuses and may require excision of the eye, other orbital contents, and involved brain. Surgery may be quite destructive and need to be repeated.
  • Pulmonary lesions can be excised if confined to a single lobe
  • Full excision of cutaneous lesions is required.
Complications

There is massive invasion and infarction of local tissues.

Prognosis

Mucormycosis has a very high mortality rate of at least 50%. Mortality from pulmonary and gastrointestinal disease is even higher due to late diagnosis. In patients who survive rhinocerebral disease, treatment requires extensive and often disfiguring facial surgery. In an Italian study 65% of diagnoses were made post mortem5 although in many empirical antifungal treatment has already been given. Mortality in transplant patients can be 80%.

Recognition is essential for early and effective treatment.Consider where there is:

  • Nasty, black discharge from the nose and palate.
  • Cranial nerve involvement causing diplopia, restricted eye movements and visual field defects.
  • Black discharge may also be seen in cutaneous variants.
  • Predisposing factors such as diabetes, malignancy or disorders of iron metabolism.

Disease of the lungs and gut is more difficult to diagnose as there are much less specific signs.


New treatments may offer better hope. A new antifungal is posaconazole: it appears to be useful for step-down therapy after initial amphotericin B treatment but it is unclear as yet if it is effective primary therapy.6


Document references
  1. Chayakulkeeree M, Ghannoum MA, Perfect JR; Zygomycosis: the re-emerging fungal infection. Eur J Clin Microbiol Infect Dis. 2006 Apr;25(4):215-29. [abstract]
  2. Safar A, Marsan J, Marglani O, et al; Early identification of rhinocerebral mucormycosis. J Otolaryngol. 2005 Jun;34(3):166-71. [abstract]
  3. Spira A, Brecher S, Karlinsky J; Pulmonary mucormycosis in the setting of chronic obstructive pulmonary disease. A case report and review of the literature. Respiration. 2002;69(6):560-3. [abstract]
  4. Lee FY, Mossad SB, Adal KA; Pulmonary mucormycosis: the last 30 years. Arch Intern Med. 1999 Jun 28;159(12):1301-9. [abstract]
  5. Pagano L, Ricci P, Tonso A, et al; Mucormycosis in patients with haematological malignancies: a retrospective clinical study of 37 cases. GIMEMA Infection Program (Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto). Br J Haematol. 1997 Nov;99(2):331-6. [abstract]
  6. Malani AN, Kauffman CA; Changing epidemiology of rare mould infections: implications for therapy. Drugs. 2007;67(13):1803-12. [abstract]

Internet and further reading Acknowledgements EMIS is grateful to Dr Chloe Borton for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2008.
DocID: 2471
Document Version: 20
DocRef: bgp471
Last Updated: 19 Mar 2008
Review Date: 19 Mar 2010

The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.

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