Definition of Mucormycosis:

Mucormycosis is a fungal infection of the sinuses, brain, or lungs that occurs mostly in people with weakened immune systems.

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 weakened immune systems are more susceptible to infection.

Conditions most commonly associated with mucormycosis include diabetes (usually poorly controlled diabetes), 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)
  • May start as a sinus infection
  • May progress to involve inflammation of cranial nerves
  • May cause blood clots that block vessels to the brain (thrombosis)
• Pulmonary mucormycosis (lung involvement) -- pneumonia that gets worse quickly and may spread to the chest cavity, heart, and brain
• Mucormycosis of the gastrointestinal tract, skin, and kidneys

Symptoms:

Symptoms of rhinocerebral mucormycosis include:

• Eye swelling and protrusion (proptosis)
• Dark nasal eschar (scabbing)
• Fever
• Headache
• Redness of skin overlying sinuses
• Sinus pain or congestion

Symptoms of lung (pulmonary) mucormycosis include:

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

Symptoms of gastrointestinal mucormycosis include:

• Abdominal pain
• Vomiting blood

Symptoms of kidney (renal) mucormycosis include:

• Fever
• Flank (side) pain

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

Signs and tests:

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

Depending on where the symptoms are, CT scans or MRIs may be done. Evaluation by an ear-nose-throat specialist is recommended if sinus involvement is suspected.

A tissue specimen must be taken and analyzed in order to make a definitive diagnosis of mucormycosis.

Treatment:

Surgery should be done immediately to remove all dead and infected tissue. Surgery can lead to disfiguration because it may involve removal of the palate, parts of the nose, or parts of the eye. Without such aggressive surgery, however, chances of survival are greatly decreased.

You will also receive antifungal medicines through a vein.

Expectations (prognosis):

Mucormycosis has an extremely high death rate even when agressive surgery is done. Death rates range from 25 - 85% depending on the body area involved and your overall health.

Complications:

• Blindness (if the optic nerve is involved)
• Clotting or blockage of brain or lung blood vessels (thrombosis)
• Death
• Nerve damage

Calling your health care provider:

People with weakened immune systems and 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.