Mucormycosis, also known as zygomycosis, is a rare fungal infection caused by the fungus Mucoromycotina (order Murcorales). Mucoromycotina were previously classified as Zygomycota. These organisms are usually found in soil and decaying organic matter, including leaves, compost or rotting wood.
Presentation
Mucormycosis is most often an acute surgical emergency, however, some cases display chronic symptoms that gradually develop over time (weeks or months). Symptoms depend heavily on which form of the disease patients have.
Symptoms of rhino-orbito-cerebral mucormycosis include acute sinusitis, fever, eye swelling and protrusion, dark nasal scabbing, cellulitis, facial pain, retinal artery thrombosis, redness of skin around sinuses and nasal congestion with black discharge. Diplopia and blindness may be observed in later stages of the disease, which indicates the infection has spread to the orbital nerves and vessels, and prognosis for these patients is poor.
Symptoms of pulmonary mucormycosis are nonspecific and may include fever, cough, rales, shortness of breath and possibly hemoptysis if tissue necrosis is present. Symptoms of GI mucormycosis include abdominal pain and distension, nausea, vomiting blood, tenderness upon palpation and hematochezia [5]. Presentation of cutaneous mucormycosis is marked by a single area of skin that is painful, hardened and may have a necrotic (blackened) central area [11]. If the mucormycosis infection has disseminated to the central nervous system patients will present with decreased consciousness and focal neurological symptoms, such as cranial nerve deficits.
Entire Body System
- Pain
Manifestations may include pain, fever, orbital cellulitis, proptosis, ophthalmoplegia, loss of vision, purulent nasal discharge, and mucosal necrosis. [msdmanuals.com]
[…] include: Cough Coughing blood (occasionally) Fever Shortness of breath Symptoms of gastrointestinal mucormycosis include: Abdominal pain Blood in the stools Diarrhea Vomiting blood Symptoms of kidney (renal) mucormycosis include: Fever Pain in the upper [nlm.nih.gov]
She denied pain, pain with eye movement, discharge or itching. She reported tearing but no foreign body sensation. She felt that her vision had been declining over the past 2 days and that her eye felt full. [eyerounds.org]
All four patients presented with some degree of headache, facial and orbital pain, with ophthalmologic symptoms ranging from pain, ptosis, and proptosis to ophthalmoplegia and blindness. [surgicalneurologyint.com]
Case Report A previously healthy 67-year-old male presented with swelling and pain over the left side of the face, deviation of the angle of the mouth to right side and drooling of saliva from left angle of mouth. [oapublishinglondon.com]
- Fever
Pulmonary symptoms (eg, productive cough, high fever, dyspnea) are severe. [msdmanuals.com]
[…] weeks Fever, RF PRED CSA MMF NIGERIAN IN USA Lurt Pakistan Enlarged graft, Hydronephrosis ureteral stenosis Graft biopsy Culture Mucor Graft Nx + Ampho-B + D 6 weeks Posaconazole Survived 20 [ 13 ] 42 M DM LRRT 1 month Fever, graft tend. [academic.oup.com]
[…] include: Cough Coughing blood (occasionally) Fever Shortness of breath Symptoms of gastrointestinal mucormycosis include: Abdominal pain Blood in the stools Diarrhea Vomiting blood Symptoms of kidney (renal) mucormycosis include: Fever Pain in the upper [nlm.nih.gov]
However, the patient then developed fever, dyspnea, and subsequent right hemiparesis. [ncbi.nlm.nih.gov]
- Intravenous Drugs
Isolated cerebral mucormycosis is a rare and serious infection associated with intravenous drug abuse. We performed a comprehensive meta-analysis of cases reported in studies and have included an unreported case from our institution. [ncbi.nlm.nih.gov]
Intravenous drug abuse. Use of voriconazole for prophylaxis or treatment of aspergillosis. [patient.info]
- Malaise
[…] incidence of rhinocerebral mucormycosis, is expected.[ 1 5 7 ] It is essential for the clinician to maintain a high index of suspicion in populations at risk, as early diagnosis can be life-saving.[ 4 ] Clinical symptoms usually begin as nonspecific malaise [surgicalneurologyint.com]
Presentation The onset of sinus mucormycosis may be associated with nonspecific symptoms such as nasal congestion, postnasal drip, dark blood-tinged or purulent rhinorrhea, sinus tenderness, headache, fever, and malaise. [aao.org]
The timeliness of definitive surgical and medical treatment is directly related to the prevention of morbidity and mortality in affected patients. 1 Case Reports Case 1 A 66-year-old man with diabetes presented to the emergency department with malaise [ajnr.org]
[…] drug use, history of transplant, and history of multiple blood transfusions.[1] [3] Initial symptoms of sinus-involving mucormycosis are consistent with acute or chronic sinusitis; congestion, blood-tinged secretions, rhinorrhea, headaches, fever, and malaise [eyewiki.aao.org]
- Fatigue
J, a 74-year-old man from Costa Rica with diabetes, was admitted to the geriatric inpatient service with a 2-week history of fatigue, weakness, and confusion, and 1 day of vomiting. [consultant360.com]
Author: Brit Long, MD (@long_brit; EM Resident at SAUSHEC; USAF) // Editor: Alex Koyfman, MD (@EMHighAK) & Justin Bright, MD (@JBright2021) A 42 year-old female with poorly controlled type II diabetes presents with several days of fatigue, headache, fever [emdocs.net]
[…] with diabetes mellitus type I, hypertension, hyperlipidemia, history of myocardial infarction, chronic myelogenous leukemia (CML), and recurrent methicillin sensitive S. aureus (MSSA) right arm cellulitis presented to a local community hospital with fatigue [clinmedjournals.org]
58 (22.4) Elevated liver laboratory tests 44 (17.1) 63 (24.3) Dyspnea 44 (17.1) 35 (13.5) Abdominal pain 43 (16.7) 59 (22.8) Headache 43 (16.7) 38 (14.7) Peripheral edema 39 (15.2) 47 (17.8) Constipation 36 (14) 54 (20.8) Insomnia 27 (10.5) 25 (9.7) Fatigue [fda.gov]
In May 2016, the patient was admitted to Affiliated Hangzhou First People's hospital again due to disease progression with fatigue and dizziness. Bone marrow pathology and chromosome karyotyping revealed MM progression and secondary MDS. [spandidos-publications.com]
Respiratoric
- Cough
Pulmonary symptoms (eg, productive cough, high fever, dyspnea) are severe. [msdmanuals.com]
Coughing blood (occasionally) Fever Shortness of breath Symptoms of gastrointestinal mucormycosis include: Abdominal pain Blood in the stools Diarrhea Vomiting blood Symptoms of kidney (renal) mucormycosis include: Fever Pain in the upper abdomen or [nlm.nih.gov]
One month before admission, she developed fever, asthenia, cough, and polyuro-polydipsic syndrome with a 10-kg weight loss. The chest X-ray was normal. [care.diabetesjournals.org]
The clinical symptoms of diabetic combined pulmonary mucormycosis included different degrees of fever, cough, sputum and dyspnea. [ncbi.nlm.nih.gov]
- Dyspnea
Pulmonary symptoms (eg, productive cough, high fever, dyspnea) are severe. [msdmanuals.com]
However, the patient then developed fever, dyspnea, and subsequent right hemiparesis. [ncbi.nlm.nih.gov]
Infusion-Related Reactions including hypotension, dyspnea, chills, dizziness, paresthesia, and hypoesthesia were reported during intravenous administration of CRESEMBA. Discontinue the infusion if these reactions occur. [cresemba.com]
- Nasal Discharge
Manifestations may include pain, fever, orbital cellulitis, proptosis, ophthalmoplegia, loss of vision, purulent nasal discharge, and mucosal necrosis. [msdmanuals.com]
Facts : Fungal infection of the sinuses, brains, lungs MCC is Rhizopus History / PE : Low grade fever Bloody nasal discharge Necrotic turbinates Diagnosis : CT / MRI imaging Treatment : Surgical debridement plus IV amphotercin B Complications : Death [medlibes.com]
Rhinocerebral mucormycosis is severe sinusitis with caused by a non- Aspergillus mold, most commonly Rhizopus arrhizus CLINICAL FEATURES Rhinocerebral mucormycosis headache facial pain confusion fever purulent nasal discharge (black) Other manifestations [lifeinthefastlane.com]
Common symptoms include sinusitis, nasal stuffiness and purulent discharge, headache, and fever. [eyerounds.org]
- Hemoptysis
Typically, pulmonary mucormycosis causes tissue necrosis resulting from angioinvasion and subsequent thrombosis, so most cases can occur with necrotizing pneumonia and/or hemoptysis. [ncbi.nlm.nih.gov]
The presence of hemoptysis should bring mucormycosis to mind because it is one of the angioinvasive fungi. [jamanetwork.com]
- Nasal Congestion
Mucormycosis Pathology Type Fungus Cause(s) Exposure to fungal spores Symptoms Clots, necrosis, headache on one side, facial pain, fever, black discharge after nasal congestion, acute sinusitis, swelling of eyes, reddening of skin, edema, difficulty breathing [house.wikia.com]
The patient also complained of nasal congestion and headache. There was no history of fever, purulent discharge, paraesthesia or foul odor. [scielo.isciii.es]
Signs of a related sinus or respiratory infection may include: cough fever headache nasal congestion sinus pain With a skin infection, mucormycosis can develop within any part of your body. [healthline.com]
Gastrointestinal
- Vomiting
We report a case of 19 year old female, with no pre-existing co-morbidities, presented with fever, dysentery, vomiting, and melena for 4 days. [ncbi.nlm.nih.gov]
Presentation is nonspecific, with abdominal pain, abdominal distension, nausea, and vomiting. [patient.info]
Symptoms of gastrointestinal mucormycosis include: Abdominal pain Nausea and vomiting Gastrointestinal bleeding Disseminated mucormycosis typically occurs in people who are already sick from other medical conditions, so it can be difficult to know which [cdc.gov]
ADVERSE REACTIONS The most frequently reported adverse reactions among CRESEMBA-treated patients were nausea (26%), vomiting (25%), diarrhea (22%), headache (17%), elevated liver chemistry tests (16%), hypokalemia (14%), constipation (13%), dyspnea (12% [cresemba.com]
- Abdominal Pain
A 14-year-old boy presented to our clinic with fever and left upper quadrant abdominal pain, and on evaluation was found to have pancytopaenia, and imaging revealed ill-defined splenic collection with thrombus in the splenic vein. [ncbi.nlm.nih.gov]
Presentation is nonspecific, with abdominal pain, abdominal distension, nausea, and vomiting. [patient.info]
Symptoms of gastrointestinal mucormycosis include: Abdominal pain Nausea and vomiting Gastrointestinal bleeding Disseminated mucormycosis typically occurs in people who are already sick from other medical conditions, so it can be difficult to know which [cdc.gov]
Symptoms of GI mucormycosis include abdominal pain and distension, nausea, vomiting blood, tenderness upon palpation and hematochezia. [symptoma.com]
- Nausea
The most commonly reported TEAEs among isavuconazole recipients were gastrointestinal disorders such as nausea, vomiting and diarrhoea. [ncbi.nlm.nih.gov]
Presentation is nonspecific, with abdominal pain, abdominal distension, nausea, and vomiting. [patient.info]
Symptoms of gastrointestinal mucormycosis include: Abdominal pain Nausea and vomiting Gastrointestinal bleeding Disseminated mucormycosis typically occurs in people who are already sick from other medical conditions, so it can be difficult to know which [cdc.gov]
(s) Exposure to fungal spores Symptoms Clots, necrosis, headache on one side, facial pain, fever, black discharge after nasal congestion, acute sinusitis, swelling of eyes, reddening of skin, edema, difficulty breathing, coughing, coughing up blood, nausea [house.wikia.com]
- Abdominal Distension
Abdominal distension and peritonitis were secondary complications. [ncbi.nlm.nih.gov]
Presentation is nonspecific, with abdominal pain, abdominal distension, nausea, and vomiting. [patient.info]
- Hematochezia
Initial manifestations may be abdominal pain and distension, fever and hematochezia. [jpgmonline.com]
Symptoms of GI mucormycosis include abdominal pain and distension, nausea, vomiting blood, tenderness upon palpation and hematochezia. [symptoma.com]
Hematochezia [24] or obstruction [25] may occur. Some patients have tenderness to palpation or a mass. Rupture may lead to signs of peritonitis. [emedicine.medscape.com]
A Rare Cause of Hematochezia: Colon Mucormycosis. Clin Gastroenterol Hepatol. 2012 Aug 28. [Medline]. Chawla N, Reddy SJ, Agrawal M. Ileocolic mucormycosis causing intestinal obstruction. Indian J Med Microbiol. 2012 Jul-Sep. 30(3):373-4. [Medline]. [emedicine.com]
Cardiovascular
- Chest Pain
There, he continued to report having chest pain. [mdedge.com]
Other symptoms include pain, warmth, excessive redness, or swelling around a wound. [cdc.gov]
Symptoms of mucormycosis that affects the lungs include cough, shortness of breath, pneumonia, fever, and chest pain. [emedicinehealth.com]
The chest X-ray was normal. Clinical assessment showed hyperthermia (38.4°C), permanent cough, hemoptysis, anterior chest pain, and crackles in the right upper field. The chest X-ray revealed a systematic opacity in the right upper lobe. [care.diabetesjournals.org]
PULMONARY MUCORMYCOSIS SEEN MOST COMMONLY IN – NEUTROPENIA,PATEINTS ON CHEMOTHERPY,LEUKEMIA. DYSPONEA ,COUGH& CHEST PAIN &FEVER RADIOLOGICALY- CONSOLIDATION,ISOLATED MASSES,CAVITAION,WEDGE SHAPED INFARCTS. CT SCAN BEST METHOD TO DETECT THE EXTENT. [slideshare.net]
Eyes
- Diplopia
Late symptoms from invasion of the orbital nerves and vessels include diplopia and visual field loss. These are late symptoms with a poor prognosis and usually are followed by reduced consciousness. [patient.info]
However, 60% to 89% of mucoceles arise from the frontal sinuses, an unusual site for mucor involvement, according to investigators. 25 Thyroid eye disease can also present with pain, diplopia, chemosis, and pressure sensation behind the eyes. [reviewofoptometry.com]
Involvement of the superior orbital fissure and its contents, such as cranial nerves III, IV, and VI, and branches of V1 and V2, may cause diplopia, ophthalmoplegia, and sensory loss to the corresponding areas of the cornea and face. [aao.org]
- Eye Swelling
Symptoms of rhinocerebral mucormycosis include: Eyes that swell and stick out (protrude) Dark scabbing in nasal cavities Fever Headache Mental status changes Redness of skin above sinuses Sinus pain or congestion Symptoms of lung (pulmonary) mucormycosis [nlm.nih.gov]
Acute sinusitis (sinus pain or congestion) Eye swelling and protrusion (proptosis) Dark nasal eschar (scabbing) Fever Redness of skin Coughing blood (occasionally) Shortness of breath fungi din ordinul Mucorales amfotericina B debridarea agresiva a leziunilor [studyblue.com]
Symptoms of rhino-orbito-cerebral mucormycosis include acute sinusitis, fever, eye swelling and protrusion, dark nasal scabbing, cellulitis, facial pain, retinal artery thrombosis, redness of skin around sinuses and nasal congestion with black discharge [symptoma.com]
- Visual Impairment
The clinical syndromes were classified as rhino-orbital, rhinocerebral or rhino-orbito-cerebral based on the presenting features such as unilateral periorbital facial pain, numbness, visual impairment/loss, eyelid edema, chemosis, proptosis, opthalmoplegia [neurologyindia.com]
- Visual Impairment
The clinical syndromes were classified as rhino-orbital, rhinocerebral or rhino-orbito-cerebral based on the presenting features such as unilateral periorbital facial pain, numbness, visual impairment/loss, eyelid edema, chemosis, proptosis, opthalmoplegia [neurologyindia.com]
Skin
- Ulcer
Cutaneous lesions are necrotic nodules that ulcerate and become covered with blackened exudates. [histopathology-india.net]
Upper gastrointestinal endoscopy revealed presence of extensive esophageal and gastric ulcer. [ncbi.nlm.nih.gov]
Another well-circumscribed ulcer of size 2 cm × 2 cm was seen near the right molar covered with yellowish black slough. The ulcers did not bleed on touch and the gums were normal [Figure 1] and [Figure 2]. [ijohsjournal.org]
Dorsal ulcer on platypus Photo: Josh Griffiths Platypus with ulcer on hind leg Photo: Nick Gust Close up of ulcer on foreleg Photo: Nick Gust These photos were taken in north central Tasmania and show platypuses with ulcerated lesions caused by Mucormycosis [dpipwe.tas.gov.au]
He was observed in stomatology for a necrotic velar ulceration that have occurred for three months. The clinical… CONTINUE READING [semanticscholar.org]
- Skin Lesion
This case highlights the importance of rapid and thorough evaluation of skin lesions when evaluating preterm infants and other immunocompromised patients, even when other sources of infection have been identified. [ncbi.nlm.nih.gov]
Figure 4 (left panel) shows an immunocompromised host with rhinocerebral mucormycosis and secondary ecchymotic skin lesions. Figure 5. [clinicaladvisor.com]
Face, Head & Neck
- Facial Pain
Also, in the case of facial pain, the low mortality rate may indicate the importance of early diagnosis. [ncbi.nlm.nih.gov]
pain sinus pressure Physical exam fevers may be only presenting symptom in immunocompromised patients with pulmonary infections soft tissue swelling black necrotic eschar on face, particularly nares or palate may affect cranial nerves blurry vision loss [step2.medbullets.com]
Rhinocerebral mucormycosis is severe sinusitis with caused by a non- Aspergillus mold, most commonly Rhizopus arrhizus CLINICAL FEATURES Rhinocerebral mucormycosis headache facial pain confusion fever purulent nasal discharge (black) Other manifestations [lifeinthefastlane.com]
Critical Essential Core Tested Community Questions (1) (M2.ID.4766) A 54-year-old male presents to the emergency department with facial pain. He reports feeling well until yesterday, when he developed a headache that has gotten progressively worse. [medbullets.com]
Symptoms of mucormycosis infection include fever, facial pain, swollen eyes, redness of skin over sinuses, bloody cough, shortness of breath, abdominal pain, vomiting blood, pain on side of the body between upper abdomen and back and a skin infection [symptoma.com]
- Facial Edema
Case report N° 2 : A 66-year-old woman with an 18-year history of diabetes mellitus presented with a 13 days history of left facial edema, headache, periorbital swelling, and fever. Palatal necrosis was noted. [latunisiemedicale.com]
Sign/symptom (%) Fever 10 (71.4) Rhinorrhea 8 (57.1) Cephalea 7 (50) Ocular pain 4 (28.6) Vision loss 3 (21.4) Palpebral edema 3 (21.4) Facial edema 2 (14.3) Proptosis 1 (7.1) 3.3. [hindawi.com]
All four patients presented with some degree of headache, facial and orbital pain, with ophthalmologic symptoms ranging from pain, ptosis, and proptosis to ophthalmoplegia and blindness. [surgicalneurologyint.com]
Differential diagnosis mainly includes necrotizing fasciitis; especially if facial edema is present [11]. [ncbi.nlm.nih.gov]
Neurologic
- Headache
After cataract surgery, he had a 3-month history of headache and pain in his right eye and on the right side of his face. [consultant360.com]
He suffered from nightly headache even was not healed with opioid drugs such as morphine and methadone. Each episode of headache has been survived about four hours accompanied by nose and eye discharge. [ncbi.nlm.nih.gov]
Symptoms of rhinocerebral mucormycosis include: Eyes that swell and stick out (protrude) Dark scabbing in nasal cavities Fever Headache Mental status changes Redness of skin above sinuses Sinus pain or congestion Symptoms of lung (pulmonary) mucormycosis [nlm.nih.gov]
- Reduced Consciousness
These are late symptoms with a poor prognosis and usually are followed by reduced consciousness. Pulmonary mucormycosis presents nonspecifically with fever, dyspneoa, and cough. [patient.info]
These late symptoms indicate a poor prognosis and are usually followed by reduced consciousness. [webmedcentral.com]
- Altered Mental Status
There was no history suggestive of visual disturbances or altered mental status. Past medical history revealed the patient to be a diabetic with a history of poor drug compliance. [ijdr.in]
Workup
Diagnosis is achieved through a variety of factors including patient’s history, risk factors and physical exam, however, a definitive diagnosis is hard to reach. The extent of infection or necrosis may be assessed through magnetic resonance imaging (MRI) or computerized tomography (CT) scan but these findings are nonspecific [12]. Biopsies can be stained with Grocott methenamine-silver stain or periodic acid-Schiff (PAS) stain and others to identify the fungus, but the specific fungal species is hard to determine [13]. More complicated cultivation and imaging techniques must be performed to identify structures unique to mucormycosis and distinguish this from other fungal infections like candidiasis and histoplasmosis. Therefore, the diagnosis of mucormycosis may be used without definitive evidence since the supportive care and treatment for many fungal infections are similar.
X-Ray
- Cavitary Lesion
Cavitary lesions with the “air crescent sign” have been described, but are rare. [omicsonline.org]
Gross specimen from the left upper lobectomy shows a 7 cm cavitary lesion surrounded by congestive and necrotic lung tissue. Fig. 5. [rc.rcjournal.com]
Imaging features in pulmonary mucormycosis are nonspecific, it can present as a solitary nodule, lobular consolidation as in pneumonia, cavitary lesion or in disseminated form 2. CT On CT, ground-glass opacities may be encountered 3. [radiopaedia.org]
So computed tomographic (CT) scan chest was done which showed a consolidation with cavitary lesion in right middle lobe ( Figure 1B ). [jtd.amegroups.com]
Treatment
Mucormycosis is treated with antifungal medications (conventional or lipid formulations of amphotericin B) administered orally or IV [14] [15]. Infected areas of skin may require surgical resection to remove infected and dead tissues [15]. Patient survival relies on proper debridement of necrotic tissue when applicable. Surgical care of rhino-orbito-cerebral infection may include repeated surgeries to excise orbital contents and infected brain tissue along with drainage of sinuses.
There are a number of potential adjunct therapies to treat mucormycosis. Hyperbaric oxygen after surgery is thought to improve neutrophil function and wound healing and inhibit fungal growth. However, the effectiveness of hyperbaric oxygen for mucormycosis has not been studies extensively and this is not a currently approved use of hyperbaric oxygen. Administration of colony stimulating factor (CSF) or interferon-gamma (IFNγ) may be done to enhance immune response in neutropenic patients and white blood cell transfusions, respectively. However, the effectiveness of these treatments is unclear. Case reports on the use of iron chelators without xenosiderophore activity, such as deferasirox, indicated that adjunct use of deferasirox leads to increased mortality rates although only a small patient sample was investigated [16].
Prognosis
The mortality rate for mucormycosis is extremely high (at least 50%). Due to the late diagnosis associated with pulmonary and GI mucormycosis infections, the mortality rate in these forms is relatively higher. Transplant patients that contract mucormycosis may experience mortality rates of 80%. An Italian study indicated that 65% of diagnoses were made postmortem [10]. The lifesaving surgery associated with rhino-orbito-cerebral mucormycosis often leaves survivors with severe facial disfigurements.
Etiology
The primary risk factor for murcormycosis is a compromised immune system. Patients with particularly high risk include those with uncontrolled diabetes mellitus, especially with ketoacidosis. Cancer patients receiving broad-spectrum antibiotics that display neutropenia, hematologic cancer patients with herpetic infection (eg. Cytomegalovirus) and graft versus host disease and individuals on immunosuppressive drugs, such as steroids and tumor necrosis factor (TNF) blockers are also at high risk for contracting murcormycosis infection.
The GI form of murcormycosis infection is linked to extreme malnutrition. The cutaneous form of murcormycosis is associated with trauma, the use of contaminated medical supplies, burns and intravenous (IV) drug use. Not all patients that develop murcormycosis will exhibit risk factors [2] [4].
Chemicals that increase chances of developing murcormycosis include iron, which is a growth stimulant for Mucorales, and deforoxamine, which is a siderophore that delivers iron to Mucorales [2] [3].
Epidemiology
Compared to other fungal infections, Mucorales infections occur much less frequently (10 to 15 fold less) [5]. Annually in the United States (US) there are an estimated 1.7 cases per million people which equates to about 500 cases per year [5].
Mucormycosis is commonly observed in immunosuppressed individuals but certain strains, such as those in the order Entomophthorales, have occurred in immunocompetent hosts. Individuals with the highest risk include those with diabetic ketoacidosis, hematologic malignancies, organ transplant recipients, chronic corticosteroid use and graft-versus-host-disease [5].
The most common form of mucormycosis is rhino-orbito-cerebral (44-49%) [7]. Cutaneous mucormycosis accounts for 10-16% of cases followed by pulmonary (10-11%), disseminated (6-11.6%) and GI (2-11%). Diabetic patients are more likely to present rhino-orbito-cerebral mucormycosis, while patients with hematological malignancies or transplants commonly present with pulmonary mucormycosis.
Outbreaks of mucormycosis have been reported including a nosocomial outbreak of GI mucormycosis that occurred in a hospital in Spain. This outbreak was linked to the use of wooden tongue depressors that were contaminated with Rhizopus and the mortality rate in this case was 40% [6].
Pathophysiology
The most common mode of entry of Mucorales is inhalation of fungal spores. Once spores enter a host they may germinate to produce hyphae that can enter blood vessels and cause thrombosis which may results in tissue necrosis. Experimentally, germinated fungal spores can adhere to the sub-endothelial matrix and cause damage to endothelial cells after phagocytosis [9]. This outcome occurs regardless of viability indicating that cidal antifungals will not be effective for the treatment of established disease [9]. Mucorales may be disseminated to other organs through the blood stream. An efficient host immune response to Mucorales infection requires normal mononuclear and polymorphonuclear phagocytes that can produce oxidative metabolites and definsins to kill fungi along with macrophages and neutrophils to inhibit spore germination and damage hyphae, respectively [8].
Factors that decrease the abundance or functioning of neutrophils, such as chemotherapy (induces neutropenia), corticosteroids, acidosis and hyperglycemia, increase the risk of developing mucormycosis [5]. Other conditions and factors that hamper the immune response to fungi include acidosis and hyperglycemia which hinder the ability of phagocytes to kill fungi and corticosteroids which prevent the ability of macrophages to stop germination [9]. Actions that result in better outcomes include early recognition and appropriate treatment along with reversal of acidosis (if it applies).
Prevention
To prevent risk of mucormycosis infection, immunosuppressants, such as corticosteroids, should be used sparingly and patients should control their diabetes. Physicians and hospitals should use appropriate rooms equipped with high-efficiency particulate air (HEPA) filters and patients with immunosuppressive conditions should use masks [5].
Summary
Several different fungal species in the order Mucorales may cause murcormycosis infections. The organisms most commonly associated with murcormycosis infection are Rhizopus. Other disease causing genera include Mucor, Cunninghamella, Apophysomyces, Absidia, Saksenaea and Rhizomucor [1] [2]. Mucormycosis infections are serious and in most cases life-threatening with disorders such as diabetic ketoacidosis and neutropenia present in most cases. Patients commonly present with severe infection of the facial sinuses which may extend to the brain (rhino-orbito-cerebral). Other infections, including pulmonary, cutaneous, gastrointestinal (GI) and disseminated, are also observed. Effective treatment of murcormycosis requires rapid correction of underlying risk factors, administration of antifungals (liposomal amphotericin B) and aggressive surgery.
Patient Information
Mucormycosis is a serious and sometimes life-threatening fungal infection that may affect the sinuses, brain, lungs and skin. The fungi that cause mucormycosis are often found in soil, decaying plants and compost and can enter the body through cuts or inhalation. Once the fungus enters the body it can spread quickly. Individuals with healthy immune systems often destroy and eliminate the fungus, however, if the immune system is weak the fungus may go on to cause a severe infection. Symptoms of mucormycosis infection include fever, facial pain, swollen eyes, redness of skin over sinuses, bloody cough, shortness of breath, abdominal pain, vomiting blood, pain on side of the body between upper abdomen and back and a skin infection that starts with blisters and later becomes tender, red, swollen and black in the center.
Patients with a compromised immune system that display the symptoms above are often diagnosed with mucormycosis. Analysis of tissue biopsies (tissue removed using small needle) is required to obtain a definitive diagnosis. Doctors may also perform CT scan or MRI to determine the extent of infection within the body and whether surgery is needed. If the central nervous system is thought to be infected patients may receive a lumbar puncture (spinal tap), if it is safe, to assess protein levels in the cerebrospinal fluid (CSF). Aggressive treatment is required if mucormycosis is suspected and early treatment is associated with better outcomes. Oral or IV antifungal medication will be administered as soon as possible and surgery may be required to remove infected and dead tissue.
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