Definition of Mucormycosis:

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 (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:

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

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 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 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:

Mucormycosis is treated right away with surgery to remove all dead and infected tissue, along with intravenous (directly into a vein) antifungal therapy. Surgical removal of infected tissue may be disfiguring because it may involve removal of the palate, parts of the nose, or parts of the eye. Withoutt his aggressive surgery, however, chances of survival are greatly decreased.

Expectations (prognosis):

Mucormycosis has an extremely high mortality rate even with aggressive surgical intervention. Death rates range from 25 - 80% depending on the body area involved and the individual's underlying immune problems.

Complications:

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

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.