Mucormycosis (Black Fungus)

Facts you should know about mucormycosis 

Mucormycosis (zygomycosis) is a serious, potentially deadly fungal infection that's infrequently diagnosed.

Many different fungi may cause mucormycosis; infections with the Mucoraceae family of fungi predominate as causes; hence, many investigators use the term mucormycosis instead of zygomycosis.

Risk factors include poorly controlled debilitating diseases (including diabetes), immunosuppression, and trauma (usually serious injuries), and groups of such patients injured in natural disasters.

Symptoms and signs first appear usually in the body area infected and may occur as follows:

fever,

headache,

reddish and swollen skin over nose and sinuses,

dark scabbing in the nose by eye(s),

visual problems,

eye(s) swelling,

facial pain,

coughing sometimes with bloody or dark fluid production,

shortness of breath,

diffuse abdominal pain,

bloody and sometimes dark vomitus,

abdominal distension,

flank pain,

an ulcer with a dark center and sharply defined edges, and

mental-status changes may occur.

Preliminary diagnosis is made by patient history, physical exam, and the patient's risk factors for mucormycosis; definitive diagnosis is made by identification of fungi in the patient's tissue.

Almost all patients require surgical debridement of infected tissue, antifungal drugs (mainly amphotericin B), and good control (treatment) of underlying medical problems such as diabetes.

Complications of mucormycosis can be dire: blindness, organ dysfunction, loss of body tissue due to infection and debridement, and death.

The prognosis (outcomes) of mucormycosis infections range from fair to poor; there is about a 50% death rate that rises to about 85% for rhinocerebral and GI infections.

Prevention of mucormycosis centers on avoidance or control of risk factors (see above), but not all infections are likely to be prevented; there is no vaccine for mucormycosis.

Research shows mucormycosis infection incidences are increasing, especially with immunosuppressed individuals. More research may occur as the number of infections increase.

Mucormycosis Symptoms & Signs

Mucormycosis is a fungal infection caused by fungi of the class Zygomycetes. These fungal infections are not common and most frequently occur in people with another form of debilitating illness. Examples of conditions that can predispose a person to developing mucormycosis include poorly controlled diabetes, decreased immune function due to any cause, and burns or severe wounds.

Symptoms and signs of the condition can vary according to the part of the body that is infected and can include

fever,

headache,

facial pain,

swelling over the nose,

facial swelling,

eye swelling,

cough,

shortness of breath,

vomiting,

abdominal pain,

flank pain,

bloody vomit,

skin ulceration,

skin swelling,

skin redness,

mental status changes,

dark scabbing in the nose,

visual problems,

bloody sputum,

abdominal distension,

skin blisters, and

coma.

What is mucormycosis?

Mucormycosis is the general term that indicates any fungal infection caused by various genera of the class Zygomycetes.

Another term used in medical and lay publications that means the same is phycomycosis.

Mucormycosis can result in an acute, rapidly advancing, and occasionally fatal disease caused by different fungi commonly found in the soil or environment. These fungal infections are diagnosed relatively infrequently; however, they occur in individual people who are debilitated in some major way (uncontrolled diabetics, immunocompromised patients) and occasionally in groups of people that are injured (often multiple injuries and penetrating injuries that are contaminated with soil and water from the environment).

Such groups of people are those that are injured in disasters such as tsunamis, hurricanes, earthquakes or tornadoes, where otherwise healthy people can have contaminated soil and water inhaled, embedded in wounds, or simply forced into skin, mouth, eyes, and nose by the force of water, soil, or wind pressure. The disease is not passed person to person.

A cluster of mucormycosis infections occurred in people who initially survived devastating tornadoes which struck Joplin, Missouri, on May 23, 2011. Thirteen cases were confirmed, all in persons with severe wounds, including fractures, multiple wounds, penetrating injuries, and blunt trauma. Ten patients required intensive care and five died.

Because the majority of mucormycosis infections are caused by one family member in the class of Zygomycetes (family member Mucoraceae), many clinicians now term the disease mucormycosis instead of zygomycosis, the more "general" term.

The lay press has used terms like "Black Death" and "Zombie disease" to describe this fungal infection but such terms seldom help people to understand this disease.

Such terms may cause misunderstandings between the patients, their families, and the public; many clinicians think these potentially harmful or cruel terms should not be used by responsible individuals.

What are risk factors for mucormycosis?

A risk factor for mucormycosis includes any debilitating disease process, especially diseases that can yield compromised blood flow to tissue.

The classic example is the patient with uncontrolled diabetes and foot ulcers where dirt or debris can easily reach compromised tissue.

Patients with burns, malignancies, immunocompromised patients, patients with a splenectomy, and people with wounds (usually severe) that have been contaminated with soil or environmental water are at higher risk to get mucormycosis.

Consequently, people injured in environmental disasters are, as a group, at high risk for this infection.

How do medical professionals diagnose mucormycosis?

Presumptive diagnosis is based on the patient's history, physical exam, and the patient's risk factors for getting a fungal infection. A definitive diagnosis is difficult.

Although tests such as CT or MRI may help define the extent of infections or tissue destruction, their findings are not specific for mucormycosis.

There are no serological or blood tests that are helpful. Growth of the fungi from a biopsy (tissue obtained by surgical removal or endoscopes with biopsy tool) of infected tissue, accompanied by special tissue stains looking for unique structural components, may identify the fungus and help make the definitive diagnosis. This helps distinguish mucormycosis from other fungal diseases such as candidiasis and histoplasmosis.

However, it is still sometimes difficult to determine the specific fungal genus and species infecting the patient.

Consequently, mucormycosis is often a "working" diagnosis that clinicians use because the supportive care and treatments for the causative fungal agents are essentially the same. Figure 2 shows a periorbital eye infection eventually diagnosed as mucormycosis.

What is the treatment of mucormycosis?

Treatments for mucormycosis need to be fast and aggressive. The need for speed is because by the time even the presumptive diagnosis is made, often the patient has suffered significant tissue damage that cannot be reversed.

Most patients will require both surgical and medical treatments.

Most infectious-disease experts say that without aggressive surgical debridement of the infected area, the patient is likely to die.

Medications play an important role. Two main goals are sought at the same time: antifungal medications to slow or halt fungal spread and medications to treat any debilitating underlying diseases.

Amphotericin B (initially intravenous) is the usual drug of choice for antifungal treatment.

In addition, posaconazole or isavuconazole may treat mucormycosis.

Patients with underlying diseases like diabetes need their diabetes optimally controlled.

Patients normally on steroids or undergoing treatment with deferoxamine (Desferal; used to remove excess iron in the body) are likely to have these medications stopped because they can increase the survival of fungi in the body.

Patients may need additional surgeries and usually need antifungal therapy for an extended time period (weeks to months) depending on the severity of the disease.

Consultation with an infectious-disease expert is advised.

What are complications of mucormycosis?

The complications of mucormycosis are serious and are related to the body area initially infected but also can occur in other body regions because the fungi often spread to the organs or tissues that physically contact or are near the originally infected area.

In addition, because surgical debridement is almost uniformly needed, some normal tissue may be destroyed because the surgeon must remove all tissue that is dead or dying.

Unfortunately, that means the surgeon may have to remove some normal tissue to insure all of the fungi are removed.

An example is infection of the eye orbit; often the whole eye must be removed.

Consequently, serious complications may occur, such as

blindness,

meningitis,

brain abscesses,

osteomyelitis,

pulmonary hemorrhages,

gastrointestinal hemorrhages,

cavitary lesions in organs and eventually secondary bacterial infections, sepsis, and death

What research is being done on mucormycosis?

Unfortunately, very little research is being done on this disease. Most studies available discuss the two major factors.

First is the data showing the increasing incidence noted in patients with debilitating diseases, currently with a focus on those who are immunocompromised by diseases or by medical treatment.

Second are studies comparing treatment plans of surgery combined with antifungal medication.

Currently, surgery and amphotericin B still seem to be the treatments that give the best results.

As the incidence and recognition of zygomycosis (mucormycosis) increases, more research may be done.