[Conidiobolomycosis (rhinofacial entomophthoromycosis) in Gabon. About of one case]. / Conidiobolomycose (entomophthoromycose rhinofaciale) au Gabon, à propos d'une observation.

Background: Entomophthoromycosis constitutes a nosological group of subcutaneous mycoses including conidiobolomycosis (rhinofacial form) and basidiobomomycosis (subcutaneous form involving the trunk and the limbs). Conidiobolomycosis is characterized by a progressive nasal and facial deformity giving, in the evolved forms, a "hippopotamus snout". The literature review finds a hundred cases, with a tropism for the humid tropical regions. Methods. We report the observation of a 25-year-old patient, living in the equatorial zone, in the south of Gabon in a humid forest area, presenting a swollen aspect of the face mainly involving the eyelids, the nose and the upper lips. Results: The diagnosis of entomophthoromycosis was compatible with the histopathological and clinical aspects. The evolution was favorable in terms of facial aesthetics under itraconazole 300 mg/day for 2 months and corticosteroid therapy (bolus of methylprednisone 240 mg/day for 3 days relayed per os at a dose of 0.5 mg/kg/day, i.e. 30 mg/day) of prednisone), maintained for 3 months. The average nasal improvement could not be completed by surgery and the patient was lost to follow-up. Conclusion: This second observation of conidiobolomycosis in Gabon in the same province makes Ngounié a privileged ecosystem for this affection.

Disseminated and ulcerative basidiobolomycosis simulating a Buruli ulcer in an immunocompetent girl in Southern Benin.

Basidiobolomycosis is a subcutaneous mycosis, for which non-specific clinical presentation can be a source of diagnostic wandering. A 5-year-old girl was brought for consultation with chronic ulcers of the pelvic limbs evolving for 8 months. The lesions started when the girl was 18 months old with a painless, pruritic nodule of the right buttock, indurated placard following progressive extension to the pelvic limbs, back and abdomen, and secondarily ulcerated in several places. On examination, there was an alteration of the general condition, a large, indurated and erythematous plaque, with sharp edges. On this plaque, there were nodular lesions and necrotic ulcers, with detached margins. The left knee was blocked in flexion. Ziehl staining and polymerase chain reaction for Mycobacterium ulcerans were negative. The histopathological picture was suggestive of basidiobolomycosis. The evolution was favorable after giving her ketoconazole (100mg per day) for 14 weeks associated with surgery and physiotherapy. This clinical case confirms the difficulties in diagnosing basidiobolomycosis, especially in endemic areas of Buruli ulcer.

Neonatal Acute Liver Failure Associated with Angioinvasive Hepatic Zygomycosis.

BACKGROUND: Neonatal acute liver failure (NALF) is often a fatal condition. Zygomycosis is a fungal infection that is often fatal in both adults and infants. Only a few cases of hepatic zygomycosis are reported in the literature and they are invariably associated with immunosuppression. MATERIALS AND METHODS: Post-mortem liver biopsy from a 14-day old neonate demonstrated confluent panacinar necrosis with angioinvasive zygomycosis. The limited work-up could not rule out an underlying immunodeficiency. CONCLUSION: Angioinvasive hepatic zygomycosis can present in the neonatal period as NALF.

Human Pathogenic Entomophthorales.

The pathogenic entomophthoralean fungi cause infection in insects and mammalian hosts. Basidiobolus and Conidiobolus species can be found in soil and insect, reptile, and amphibian droppings in tropical and subtropical areas. The life cycles of these fungi occur in these environments where infecting sticky conidia are developed. The infection is acquired by insect bite or contact with contaminated environments through open skin. Conidiobolus coronatus typically causes chronic rhinofacial disease in immunocompetent hosts, whereas some Conidiobolus species can be found in immunocompromised patients. Basidiobolus ranarum infection is restricted to subcutaneous tissues but may be involved in intestinal and disseminated infections. Its early diagnosis remains challenging due to clinical similarities to other intestinal diseases. Infected tissues characteristically display eosinophilic granulomas with the Splendore-Höeppli phenomenon. However, in immunocompromised patients, the above-mentioned inflammatory reaction is absent. Laboratory diagnosis includes wet mount, culture serological assays, and molecular methodologies. The management of entomophthoralean fungi relies on traditional antifungal therapies, such as potassium iodide (KI), amphotericin B, itraconazole, and ketoconazole, and surgery. These species are intrinsically resistant to some antifungals, prompting physicians to experiment with combinations of therapies. Research is needed to investigate the immunology of entomophthoralean fungi in infected hosts. The absence of an animal model and lack of funding severely limit research on these fungi.

Rhinofacialentomophthoromycosis.

Entomopthoromycosis is a rare subcutaneous fungal infection caused by onidiobolus coronatus affecting mainly the upper respiratory mucosa in immunocompetent people.The manifestations of this disease masquerades other clinical entities.Hence, high index of suspicion is required for prompt diagnosis.Histopathological examination and culture are the gold standard diagnostic tools, however no standard treatment protocols ha been mentioned in literature.We present a case of rhinofacial entomopthoromycosis in a yearold ma with a leftsided rhinofacial swelling to highlight the presence of this unusual fungal infection and its treatment.

Gastro-intestinal basidiobolomycosis in a 2-year-old boy: dramatic response to potassium iodide.

Gastro-intestinal basidiobolomycosis (GIB) is a rare fungal infection caused by Basidiobolus ranarum. Treatment includes surgical resection and long-term antifungal therapy. A 2.5-year-old boy presented with a 10-day history of abdominal pain, fever and diarrhoea, and a palpable abdominal mass was detected. Resection was undertaken and histology confirmed basidiobolomycosis. Treatment with amphotericin B and itraconazole was commenced, but the infection progressed and spread to involve the intestines, liver, ribs and lung, and also the abdominal wall after 6 months, requiring four operative procedures. Because of unresponsiveness to amphotericin and itraconazole, oral potassium iodide was added which resulted in complete resolution of the infection. Potassium iodide is an essential component of the treatment of systemic B. ranarum.

Successful Treatment of Invasive Conidiobolus Infection During Therapy for Acute Lymphoblastic Leukemia.

Invasive fungal infections are a serious cause of morbidity and mortality in patients with hematologic malignancies. Conidiobolus species are molds within the order Entomophthorales and may disseminate to become rapidly fatal in immunocompromised individuals. This species of fungal infections are often multidrug resistant (MDR) and present unique therapeutic challenges. Reports of Conidiobolus infections are rare in pediatric oncology. We report the successful treatment of an adolescent male with B-cell lymphoblastic leukemia and MDR invasive sinopulmonary Conidiobolus infection with emphasis on early and aggressive neutrophil support with surgical debridement. The strategies described could be applied to other MDR fungal infections.

Invasive Conidiobolomycosis Can Be Successfully Treated on Burn Survivors.

Conidiobolus infection is difficult to diagnose and treat. We report successful treatment of invasive conidiobolomycosis, only one third such report is in the literature, and the first case reported was on a burn survivor. Our patient is also the first case reported on an adult surviving conidiobolomycosis. This case illustrates that serum ß-D-glucan (BDG) may be useful in the diagnosis and monitoring of invasive Conidiobolus infection, contrary to the misconception that Zygomycetes do not release detectable levels of BDG. Aggressive, surgical management and care in the burn unit can lead to successful treatment of this otherwise lethal infection.

Epidemiological aspects and clinical outcome of patients with Rhinocerebral zygomycosis: a survey in a referral hospital in Iran.

INTRODUCTION: No comprehensive reports have been published on epidemiological status of Rhinocerebral zygomycosis infections and its outcome in our population, Hence, the current study came to address epidemiological characteristics as well as clinical outcome of patients with Rhinocerebral zygomycosis infection referred to a referral hospital in Iran. METHODS: This retrospective study was performed at the Rasoul-e-Akram hospital, an 800-bed tertiary care teaching hospital in Tehran, Iran. The pathology recorded charts were reviewed to identify all cases of Rhinocerebral zygomycosis from patients admitted between April 2007 and March 2014. A diagnosis of Rhinocerebral zygomycosis was based on histopathological assessments. RESULTS: Sixty four patients with Rhinocerebral zygomycosis were assessed. The mean age of the patients was 46.07 ± 22.59 years and 51.6% were female. Among those, 67.2% were diabetic, 26.6% were hypertensive and 29.7% had history of cancer. Different sinuses were infected in 73.4% of the patients. Out of all the patients 26.6% underwent surgical procedures and 17.2% were controlled medically. Extensive debridement was carried out in 40.6%. Neutropenia (<1500 cell/ µl) was revealed in 12.5%. In-hospital mortality rate was 35.9% and prolonged hospital stay (> 14 days) was found in 60.9%. According to the Multivariable logistic regression analysis, the main predictors of in-hospital mortality included female gender, advanced age, the presence of sinus infection, and neutropenia, while higher dosages of amphotericin administered had a protective role in preventing early mortality. In a similar Multivariate model, history of cancer could predict prolonged hospital stay, whereas using higher dose of amphotericin could lead to shortening length of hospital stay. CONCLUSION: There is no difference in demographic characteristics between our patients with Rhinocerebral zygomycosis and other nations. The presence of diabetes mellitus is closely associated with the presence of this infection. Sinus involvement is very common in those with Rhinocerebral zygomycosis leading to high mortality and morbidity. Besides female gender, advanced age, and presence of neutropenia was a major risk factor for increasing early mortality. The use of higher doses of antifungal treatment such as amphotericin can prevent both mortality and prolonged hospital stay. The cancer patients may need longer hospital stay because of needing comprehensive in-hospital treatment.

[Clinical analyses of the diagnosis and treatment of invasive fungal rhinosinusitis: report of 14 cases].

OBJECTIVE: Through the retrospective analysis of the clinical data in 14 cases of invasive fungal rhinosinusitis (IFRS), the clinical characteristics, diagnosis and treatment of this disease were evaluated. METHODS: Fourteen clinically confirmed cases of IFRS since January 2008 to October 2015 were evaluated.collected, the clinical features, diagnosis, treatment and prognosis were analyzed to obtain a more comprehensive understanding for clinical reference. Fourteen patients were confirmed by pathological examination as IFRS, including 9 cases of aspergillus, 4 cases of mucor, and 1 case of rhinocerebral zygomycosis; including 5 cases of acute IFRS, 9 cases of chronic IFRS. All patients were treated with endoscopic surgery and intravenous antifungal therapy. RESULTS: Nine cases of chronic IFRS (including 1 case of mucor, 7 cases of aspergillus and 1 case of rhinocerebral zygomycosis) were cured, but the vision loss, diplopia or blindness, hard palate perforation remained. Five cases of acute IFRS included 3 cases of mucor and 2 cases of aspergillus. Among the 3 cases of mucor, 2 cases were died and 1 case was cured. Among the 2 cases of aspergillus, 1 patient was cured and the other patient died of electrolyte disorder after discharge from hospital. CONCLUSIONS: Patients with IFRS usually have diabetes. After the active surgical cleaning of lesion tissue and the systematic antifungal treatment with adequate dosage, these patients would have a better result. IFRS caused by mucor is ofen dangerous.

Entomophthoramycosis: a neglected tropical mycosis.

The term 'entomophthoramycosis' classically refers to infections caused by members of the order Entomophthorales. A new subphylum, Entomophthoramycota, has been created to include Basidiobolomycetes, Neozygitomycetes and Entomophthoramycetes. Basidiobolomycetes encompass Basidiobolus spp., while the Entomophthoramycetes include Conidiobolus spp. Conidiobolus spp. characteristically cause rhinofacial entomophthoramycosis in apparently immunocompetent hosts. Conidiobolus spp. may also cause disseminated infection in immunocompromised patients. Basidiobolus spp. more typically cause subcutaneous entomophthoramycosis of the limbs, buttocks, back and thorax in immunocompetent patients. While once considered to be rare, there is an increasing number of reported cases of gastrointestinal infection caused by Basidiobolus spp. worldwide in countries such as United States, Thailand, Australia, Iran, Egypt and Saudi Arabia. These cases have clinical presentations similar to those of inflammatory bowel diseases, particularly Crohn's disease. Retroperitoneal, pulmonary, nasal and disseminated basidiobolomycosis have also been reported. Histology of entomophthoramycosis may reveal the Splendore-Hoeppli phenomenon. Culture of infected tissue remains the definitive method of laboratory diagnosis. However, molecular methods with specific DNA probes and panfungal primers, as well as real time PCR, are increasingly used to detect and identify these organisms in tissue. Treatment largely consists of therapy with antifungal triazoles. Surgery plays a selective role in the management of entomophthoramycosis, depending upon location, organism and extent of the infection.

Acute cutaneous zygomycosis of the scalp: A case report and literature review.

Cutaneous zygomycosis is the third most common form of zygomycosis. However, scalp involvement is rare for this disease. In this study, we present a case of acute zygomycosis in a diabetic patient who was effectively treated with local debridement, amphotericin B lipid complex and posaconazole.

Subcutaneous entomophthoromycosis mimicking soft-tissue sarcoma in children.

AIM: Subcutaneous entomophthoromycosis (EM) is an uncommon fungal infection of childhood. This article is intended to draw the attention of pediatric surgeons to the fact that EM can mimic soft-tissue tumor. METHODS: It is a retrospective review of 16 children treated for subcutaneous EM between 2000 and 2013. RESULTS: The median age of patients was 3.5 years. The typical lesion was a discoid subcutaneous mass that can be easily lifted from deeper tissues (the doughnut lifting sign). Lesions were mostly distributed in the lower half of body. All the patients were immunocompetent. Correct clinical diagnosis was made only in 4 cases while others were mistaken for a tumor. All the 8 children who underwent wide excision of the pseudotumor had local recurrence. Supersaturated solution of potassium iodide was curative in 11 cases while addition of itraconazole was needed in one case. One child died of multi-drug resistant infection. The mean treatment duration was 4.7 months (range 2-8 months). CONCLUSION: Subcutaneous EM can mimic soft-tissue tumor. High index of suspicion is essential to avoid misdiagnosis and inappropriate treatment. A newly described "doughnut-lifting sign' may be helpful in clinical diagnosis. Emergence of multi-drug resistant infection is a source of concern.

Reno-invasive fungal infection presenting as acute renal failure: importance of renal biopsy for early diagnosis.

Renal zygomycosis, caused by invasive fungi, is a rare and potentially fatal infection. The patient usually presents with non-specific symptoms and renal failure. A 34-year-old male non-diabetic and without any predisposing factors for systemic fungal infection presented to the emergency department with diffuse abdominal pain, high-grade fever and acute renal failure with a serum creatinine of 6.5. A computed tomography showed bilateral diffuse globular nephromegaly. A urine smear for fungal examination showed right angle branching hyphae and kidney biopsy showed fungal hyphae within the glomeruli, tubules and interstitium. Although radiological investigations can give us a clue, the definitive diagnosis can only be made by kidney biopsy. A high index of suspicion and timely diagnosis is important for a proper management.

Central nervous system mycosis: analysis of 10 cases.

AIM: To describe the clinicopathological features in patients with fungal infections of the central nervous system (CNS) presenting as mass lesions. MATERIALS AND METHODS: A retrospective analysis of records obtained from 10 patients was done with histopathologically confirmed fungal infections presenting as ICSOL, diagnosed in the department of pathology. Clinical features at presentation, findings of radiological investigations performed and histopathology were noted for each patient and subjected for analysis. RESULTS: Infection was higher in males, and paranasal sinusitis was the most common predisposing factor. Location was intraparenchymal followed by sphenoid wing. Four dural-based lesions mimicked meningioma clinically. The most common fungus identified was zygomycosis (seven cases), followed by phaeohyphomycosis (two cases) and aspergillosis (one case). CONCLUSION: There is a rising trend of CNS mycosis, both in immunocompromised and immunocompetent patients. Intracranial fungal granuloma may mimic radiologically as glioma or meningioma, therefore a high index of suspicion is needed to detect early CNS fungal infections, especially in immunocompetent young patients with no predisposing illness. Fungi should always be excluded in patients with inflammatory or granulomatous pathology of CNS.

[Pulmonary zygomycosis in a diabetic child complicated with thrombus of the left atrial auricle]. / Mucormycose pulmonaire chez une enfant diabétique, compliquée d'un thrombus intra-auriculaire gauche.

Mucormycosis is a rare but fatal, opportunistic fungal infection caused by fungi of the order of mucorales in the class of Zygomycetes. Isolated pulmonary mucormycosis is rare and occurs principally in particular conditions, especially in patients with uncontrolled diabetes. The fungi invades the blood vessels and causes distal ischemic necrosis. We report a case in a 13-year-old girl with diabetes mellitus who developed pulmonary zygomycosis complicated with thrombus of the left atrial auricle. The diagnosis of mucormycosis was confirmed by histologic examination of endobronchial biopsies. Combined treatment with systemic amphotericin B and surgery resulted in a favorable outcome. This difficult diagnosis must be raised in diabetic patients with unresponsive lung infections in order to apply early aggressive therapy. Successful management continues to be early diagnosis, followed by systemic antifungal therapy and surgical resection combined with control of the underlying disease.

Zigomicose rino facial: relato de caso / Rhino facial zygomycosis: case report

A zigomicose é uma doença invasiva, que acomete tanto imunocompetentes como imunocomprometidos, dependendo do tipo da cepa. O diagnóstico é clínico e histopatológico, e o tratamento é baseado em antifúngico e em limpeza cirúrgica. O presente relato de caso é sobre um menino com zigomicose rinofacial invasiva com tratamento final bem-sucedido, após terapias antifúngicas e limpezas cirúrgicas.

Zygomycosis is an invasive disease that affects both immunocompetent and immunocompromised, depending on the type of strain. This disease diagnosis is clinical and histopathological, and its treatment is based on antifungal therapy and surgical cleaning. This paper reports a case of a boy with invasive zygomycosis rinofacial who final treatment was successful after underwent antifungal and surgical therapies.

Rhino facial zygomycosis: case report.

Zygomycosis is an invasive disease that affects both immunocompetent and immunocompromised, depending on the type of strain. This disease diagnosis is clinical and histopathological, and its treatment is based on antifungal therapy and surgical cleaning. This paper reports a case of a boy with invasive zygomycosis rinofacial who final treatment was successful after underwent antifungal and surgical therapies.