Mucormycosis was formerly also known as cyclomycosis. It is the third most common fungal infection after candidiasis and aspergillosis. The disease mainly occurs in people with an immune deficiency.
What is mucormycosis?
Mucormycosis is a fungal infection with a fulminant course. It is caused by fungi from the Zygomycetes family. Normally, the zygomycetes belong to the saprophytes. See foodezine for Everything about Heart Attack.
Saprophytes feed exclusively on dead organic matter. In the event of an immune deficiency, the actually harmless saprophytes can become parasites and cause serious damage to the body. The rhinocerebral form of the disease is particularly feared. The fungi enter the brain via the paranasal sinuses and cause severe damage there.
Causes
The causative agents of mucormycosis are filamentous fungi that grow in a filamentous manner. Since they are usually harmless, but can cause serious damage under certain circumstances, they belong to the facultative pathogenic fungi. The most common pathogens of mucormycosis are fungi of the genera Mucor, Rhizomucor, Rhizopus, Lichtheimia and Cunninghamella. The fungi are distributed worldwide (ubiquitously) and are mainly found in the soil.
Normally humans are immune to these fungi. With a weakened immune system, however, they can spread in the respiratory tract, in the gastrointestinal tract or on the skin. The pathogens penetrate the tissue and blood vessels very quickly. Particularly predisposed are patients with diabetic ketoacidosis, patients after stem cell or organ transplantation, patients receiving corticosteroid therapy or people who have severe burns. Patients with T-cell defects or an advanced HIV infection are also particularly susceptible to mucormycosis.
Symptoms, Ailments & Signs
Five forms can be distinguished in mucormycosis. All are life threatening:
- The most common form is rhinoorbitocerebral mucormycosis. It is mainly found in children with diabetes mellitus or in leukemia patients. Characteristic symptoms of this form are redness, pain and swelling in the face and eye sockets. The infection starts in the paranasal sinuses and causes sinusitis there. The nasal discharge is bloody. There are also black tissue lesions in the nasal mucosa.
The thread-like extensions of the fungi break through the skin and grow into the tissue and bones, so that soft facial tissue, the eye socket, the meninges and the frontal lobe can also be affected. Damage to the arteries and veins also increases the risk of thrombosis and heart attacks. Alterations in consciousness, central paralysis, and visual disturbances indicate central nervous system involvement.
- In the case of pulmonary mucormycosis, the lungs in particular are affected by the fungi. Here, too, thrombosis and infarction occur. These are often accompanied by fever, shortness of breath and chest pain. Cancer patients with acute leukemia are particularly affected. Infection can arise from rhinoorbitocerebral mucormycosis. As a rule, however, it develops directly in the lungs after inhaling the fungal spores.
- Disseminated mucormycosis is particularly common in children with acute leukemia. It starts in the lungs and then spreads to other organs and the central nervous system via the bloodstream. Disseminated mucormycosis is almost always fatal.
- Primary gastrointestinal mucormycosis is diagnosed less frequently. It is characterized by ulcers in the gastrointestinal tract and occurs predominantly in immature newborns. The ulcers are at risk of perforation. The penetration of the pathogens into the blood vessels can also lead to infarctions here.
- Skin mucormycosis is seen in patients with severe burns or in leukemia patients. Black necrosis of the skin is typical of this form.
Diagnosis & course of disease
The clinical and radiographic findings are similar to those of other fungal infections. The diagnosis can therefore only be made with the help of cultural, microscopic or histopathological evidence of the pathogen. Diagnostic material can be obtained through biopsies or surgical procedures.
Possible procedures for obtaining material are skin or soft tissue biopsies, endoscopies of the nose and paranasal sinuses, bronchoscopy with lavage or CT – guided biopsies. In the case of rhinocerebral mucormycosis, diagnostic imaging also shows thickening of the mucous membrane in the area of the paranasal sinuses. Pulmonary mucormycosis shows nonspecific and extensive lesions of the lungs in the X-ray image. Patchy infiltrations, meltdowns or pleural effusions may be visible.
If the orbit is affected, a pathological mass appears there. In addition to detecting the pathogen, detailed imaging using computer and magnetic resonance tomography is always required. This is the only way to capture the full extent of the infection.
Complications
Various symptoms can occur in patients with mucormycosis. As a rule, this disease must be treated by a doctor in any case, as it can be life-threatening and, in the worst case, lead to the death of the patient. Those affected suffer primarily from severe swelling in the face and also in the eye socket.
It comes to nosebleeds and also to changes in consciousness. Normal thinking and acting is only possible with difficulty for those affected and most patients suffer from severe visual disturbances. Without treatment, fever and shortness of breath occur, which can lead to loss of consciousness. Chest pain can also occur.
Ulcers form in the intestines or stomach, which can also lead to death. The quality of life decreases significantly as a result of mucormycosis and everyday life is made considerably more difficult for the patient. Mucormycosis is treated with medication or chemotherapy. Whether this will lead to a positive course of the disease cannot be predicted. In many cases, life expectancy is significantly reduced by mucormycosis.
When should you go to the doctor?
A mucormycosis should be clarified by a doctor at an early stage. At the latest when typical symptoms such as skin changes on the palate and infections in the nose and throat are noticed, a doctor must be consulted. Otherwise, further expansion of the necrosis may occur. Signs such as seizures, aphasia or hemiplegia indicate an advanced disease that needs to be investigated immediately. People with chronic infectious diseases, recurring infections or a generally weakened immune system are particularly susceptible to the development of mucormycosis and should see a doctor as soon as possible if their state of health deteriorates noticeably.
The same applies to elderly and sick people, pregnant women and children. In addition to the family doctor, there are ENT doctors and specialists in infectious diseases. Children should always be presented to the responsible pediatrician first. If health problems arise again after the treatment, the doctor responsible must be informed due to the risk of a recurrence. Constant medical monitoring is also indicated during the treatment of mucormycosis.
Treatment & Therapy
The therapy of mucormycosis is multimodal. The cornerstone is always antifungal chemotherapy. In addition, an attempt is made to eliminate the underlying immunological or metabolic defect. This forms the breeding ground for the fungi. If the underlying disease is not eliminated, the fungi will spread again after chemotherapy. Treatment is supplemented with antifungal drugs such as amphotericin B.
The duration of therapy depends on the extent of the mucormycosis. Depending on age, pathogen and underlying disease, the mortality rate is between 50 and 70 percent. Patients only have a chance of survival if they receive consistent therapy. The prognosis is worsened by disseminated infections, cancer as the underlying disease, and diseases associated with a lack of granulocytes. Once the central nervous system has been reached, the disease is almost always fatal.
Outlook & Forecast
In principle, an unfavorable outcome can be assumed for mucormycosis. According to statistical surveys, 50 to 70 percent of those infected die. The risk of premature death is distributed differently. It increases with other underlying diseases and old age. If symptoms have spread to the central nervous system, death is usually inevitable. In general, people with a weakened immune system and a metabolic disease are considered to be comparatively susceptible to mucormycosis. In them, the disease is comparatively severe and usually leads to life-threatening complications.
The therapeutic approaches available so far are mostly inadequate. It is precisely this fact that causes the high mortality rate. In any case, only consistent therapy can contribute to recovery. Starting treatment early promises better prospects. In practice, it often turns out to be problematic that an exact diagnosis is not possible. In many cases, therefore, treatment is started on mere suspicion. Only after death can the disease be determined. In the recent past, science has developed molecular-biological methods for diagnosis. Improvements can be expected from this.
Prevention
So far there is no effective and specific prophylaxis for mucormycosis. Mucormycoses in children or adolescents with diabetes mellitus can be avoided by optimal blood sugar control. A permanently elevated blood sugar level impairs the immune system and thus promotes fungal infections.
Aftercare
In most cases, those affected with mucormycosis have very few measures and options for direct follow-up care. For this reason, the affected person should consult a doctor as early as possible in order to prevent the recurrence of symptoms and complications. As a rule, mucormycosis cannot heal on its own.
Most of those affected are dependent on the help and care of their own families. In many cases, this can also prevent depression and other psychological complaints or upsets. Furthermore, it is not uncommon for various medications to be taken in order to completely limit the symptoms of this disease.
Those affected should always ensure that they are taken regularly and that the dosage is correct in order to relieve the symptoms permanently and, above all, correctly. As a rule, the person concerned should also protect themselves particularly well against infections. Vaccinations should also be carried out so that various diseases cannot occur.
In some cases, mucormycosis also reduces the patient’s life expectancy. However, the further course depends heavily on the time of diagnosis, so that a general prediction cannot usually be made.
You can do that yourself
Mucormycosis must always be examined and treated by a doctor. The medical therapy can be supported by a change in lifestyle and various self-help measures.
During radiation or chemotherapy, the patient must take it easy. A special diet reduces the typical gastrointestinal complaints and promotes the reduction of metastases. At the same time, any allergies must be clarified and medication taken must be checked and adjusted if necessary. Patients who regularly use headache tablets or other medicines should inform the doctor about this. Optimally adjusted medication reduces the risk of complications and can also promote recovery. If thrombosis, visual disturbances, paralysis or other problems have already set in as a result of the mucormycosis, these must be treated separately. The doctor will refer the patient to a specialist.
The most important self-help measure is to keep a log of complaints and symptoms, because this allows the stage of mucormycosis to be determined precisely and the treatment to be chosen optimally. In addition, serious complications can be recognized and treated early, before a life-threatening condition sets in. Since the fungal infection is a protracted disease, close medical monitoring is indicated beyond the initial treatment.