Mucormycosis

Alternative names
Zygomycosis

Definition
Mucormycosis is a fungal infection of the sinuses, brain, or lungs that occurs primarily in people with immune disorders.

Causes, incidence, and risk factors

Mucormycosis is caused by common fungi frequently found in the soil and in decaying vegetation. Most individuals are exposed to these fungi on a daily basis - but people with immune disorders may be more susceptible to infection.

Conditions most commonly associated with mucormycosis include diabetes mellitus, chronic steroid use, metabolic acidosis, organ transplantation, leukemia/lymphoma, treatment with deferoxamine, and AIDS.

Syndromes associated with mucormycosis include:

     
  • Rhinocerebral infection (infection of sinuses and brain)       o May start as a sinus infection       o May progress to involve inflammation of cranial nerves       o May cause blood clots that block vessels to the brain (thrombosis)  
  • Pulmonary mucormycosis (lung involvement) - rapidly progressive pneumonia that may spread to the chest cavity, heart, and brain.  
  • Mucormycosis of the gastrointestinal tract, skin, and kidneys.

Symptoms

Symptoms of rhinocerebral mucormycosis include the following:

     
  • Acute sinusitis  
  • Fever  
  • Eye swelling and protrusion of eye orbit (proptosis)  
  • Dark nasal eschar (scabbing)  
  • Redness of skin overlying sinuses

Symptoms of pulmonary mucormycosis include the following:

     
  • Fever  
  • Cough  
  • Coughing blood (occasionally)  
  • Shortness of breath

Symptoms of gastrointestinal mucormycosis include the following:

     
  • Abdominal pain  
  • Vomiting blood

Symptoms of renal mucormycosis include the following:

     
  • Flank pain  
  • Fever

Symptoms of cutaneous mucormycosis include a single, painful, hardened area of skin that may have a blackened central area.

Signs and tests

Mucormycosis should be suspected if symptoms appear in individuals with immune disorders such as diabetes or those with weakened immune systems such as transplant recipients. Symptoms of rhinocerebral mucormycosis are most likely to occur among immunosuppressed people.

Depending on the site of involvement, CT scans or MRIs may be performed. Evaluation by an ear-nose-throat specialist is recommended if sinus involvement is suspected.

To definitively diagnose mucormycosis, a tissue specimen must be obtained and analyzed.

Treatment

The mainstay of treatment for mucormycosis is early surgical intervention to remove all dead and infected tissue, along with intravenous antifungal therapy. Surgical removal of involved tissue is critical and may be disfiguring because it may involve removal of the palate, nasal structures, or eye structures.

However, chances of survival are greatly decreased without this aggressive intervention.

Expectations (prognosis)
Mucormycosis has an extremely high mortality rate even with aggressive surgical intervention. Death rates range from 25-80% depending on the site involved as well as the underlying immune problems.

Complications

     
  • Loss of neurological function (nerve involvement)  
  • Clotting/blockage of brain or lung vessels (thrombosis)  
  • Blindness (if the optic nerve is involved)

Calling your health care provider
People with immune disorders (including diabetes) should seek medical attention if they develop fever, headache, sinus pain, eye swelling, or any of the other symptoms listed above.

Prevention
Because the fungi that cause mucormycosis are widespread, the most appropriate preventive measures involve improved control of the underlying illnesses associated with mucormycosis.

Johns Hopkins patient information

Last revised: December 3, 2012
by Gevorg A. Poghosian, Ph.D.

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