Entomoftoromicosis por Conidiobolus coronatus en un paciente joven proveniente de área rural / Entomophthoromycosis due to Conidiobolus coronatus in a young patient from a rural area

La conidiobolomicosis es una micosis subcutánea causada por un hongo saprofito, Conidiobulus spp. perteneciente a la clase Zigomicetos, orden Entomoftorales, que habita en regiones tropicales. La manifestación clínica clásica es la deformidad progresiva de estructuras faciales y su diagnóstico se basa en cultivos de la zona afectada y el estudio histopatológico, siendo el "fenómeno de Splendore-Hoeppli" el hallazgo más característico. Dada su baja frecuencia de presentación, no existe consenso sobre la mejor opción y tiempo de tratamiento. Aquí presentamos un caso de entomoftoromicosis rinofacial causada por Conidiobolus coronatus en un paciente inmunocompetente de la región sur de Colombia.

Conidiobolomycosis is a subcutaneous mycosis caused by a saprophytic fungus, Conidiobulus, belonging to the class of Zygomycetes, an order of Entomophtorales that inhabits tropical regions. Its most frequent clinical manifestation is the progressive deformity of facial midline structures, and the diagnosis is based on cultures taken from the affected area and the histopathological study, being the "Splendore-Hoeppli phenomenon" the most characteristic finding. Due to its low frequency of presentation, there is no consensus about the best option and treatment time. We present a case of rhinofacial entomophthoromycosis caused by Conidiobolus coronatus in an immunocompetent patient from the southern region of Colombia.

Rhinofacial Conidiobolomycosis: A Case Series and Review of the Literature.

Conidiobolomycosis is an uncommon, chronic, localized subcutaneous mycosis primarily affecting rhinofacial region. It is reported mainly from tropical and subtropical countries. The condition is underreported due to the lack of clinical suspicion and usually mismanaged. This rare mycosis is due to the genus Conidiobolus within the order Entomophthorales of class Zygomycetes. Here we present 3 cases of rhinofacial conidiobolomycosis in otherwise healthy adults from different parts of Sri Lanka over 1-year period. All patients had disfiguring subcutaneous lesions in the rhinofacial area. The diagnoses were based on isolation of Conidiobolus coronatus in clinical specimens.

Conidiobolus pachyzygosporus invasive pulmonary infection in a patient with acute myeloid leukemia: case report and review of the literature.

BACKGROUND: Conidiobolus spp. (mainly C. coronatus) are the causal agents of rhino-facial conidiobolomycosis, a limited soft tissue infection, which is essentially observed in immunocompetent individuals from tropical areas. Rare cases of invasive conidiobolomycosis due to C. coronatus or other species (C.incongruus, C.lamprauges) have been reported in immunocompromised patients. We report here the first case of invasive pulmonary fungal infection due to Conidiobolus pachyzygosporus in a Swiss patient with onco-haematologic malignancy. CASE PRESENTATION: A 71 year-old female was admitted in a Swiss hospital for induction chemotherapy of acute myeloid leukemia. A chest CT performed during the neutropenic phase identified three well-circumscribed lung lesions consistent with invasive fungal infection, along with a positive 1,3-beta-d-glucan assay in serum. A transbronchial biopsy of the lung lesions revealed large occasionally septate hyphae. A Conidiobolus spp. was detected by direct 18S rDNA in the tissue biopsy and subsequently identified at species level as C. pachyzygosporus by 28S rDNA sequencing. The infection was cured after isavuconazole therapy, recovery of the immune system and surgical resection of lung lesions. CONCLUSIONS: This is the first description of C. pachyzygosporus as human pathogen and second case report of invasive conidiobolomycosis from a European country.

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.

Gastrointestinal pythiosis with concurrent presumptive gastrointestinal basidiobolomycosis in a Boxer dog.

A 2-year-old female spayed Boxer dog was presented for a 1-month history of progressive hemorrhagic diarrhea with tenesmus and weight loss despite trial courses of antibiotics and diet change. Abdominal ultrasound revealed severe, focal thickening, and loss of normal architecture of the colonic wall with abdominal lymphadenomegaly. Dry-mount fecal cytology, performed on several consecutive days, consistently revealed numerous, round, 16-20 µm structures with basophilic, granular content, and a thin cell wall. Transmission electron microscopy identified these structures as fungi. Culture, polymerase chain reaction (PCR), and sequencing of the internal transcribed spacer, D1/D2 regions, and DNA-directed RNA polymerase II core subunit (RPB2) confirmed the presence of Basidiobolus microsporus in the feces. Biopsies collected via ileocolonoscopy revealed marked, multifocal, chronic, neutrophilic, and eosinophilic ileitis and colitis with ulceration, granulation tissue, and intralesional hyphae (identified with Gomori methenamine silver stain). A Pythium enzyme-linked immunosorbent assay and Pythium-specific PCR performed on the formalin-fixed paraffin-embedded biopsy specimens were positive while Basidiobolus-specific PCR was negative, thus confirming a diagnosis of pythiosis. This report describes a fatal case of colonic and intestinal pythiosis with the presence of fecal Basidiobolus sp. spores, suggestive of concurrent gastrointestinal basidiobolomycosis.

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.

Rhinocerebral Zygomycosis Due to a Lichtheimia ramosa Infection in a Calf: Neural Spread Through the Olfactory Nerves.

Here, we report a case of rhinocerebral zygomycosis due to a Lichtheimia ramosa infection in a calf. A histopathological examination revealed that a fungus had invaded the brain through the olfactory nerves. Lichtheimia ramosa was detected by polymerase chain reaction analysis of DNA extracted from formalin-fixed paraffin-embedded samples of the affected tissue. This is the first case of rhinocerebral zygomycosis to involve cattle. Also, this is the first such case to involve fungal invasion into the central nervous system through the cranial nerve itself, rather than through perineural tissue.

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.

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.

Colonic basidiobolomycosis with liver involvement masquerading as gastrointestinal lymphoma: a case report and literature review.

Basidiobolomycosis is an unusual fungal skin infection that rarely involves the gastrointestinal tract. This study reported a 5-year-old boy with gastrointestinal basidiobolomycosis that had been misdiagnosed as gastrointestinal lymphoma. He was treated by surgical resection and a combination of posaconazole and amphotericin B deoxycholate with an acceptable response and no recurrence.

Subcutaneous basidiobolomycosis: A Case Report.

Basidiobolomycosis is an uncommon chronic deep fungal infection in which gradually enlarging granulomas form, usually in the subcutaneous fat tissues of the limbs, chest or trunk of immunocompetent hosts, primarily children and young adults. It is caused by the fungus Basidiobolus ranarum. Definitive diagnosis is by microscopy and histopathology. Effective treatments include ketoconazole, itraconazole, potassium iodide and co-trimoxazole. A 3 year old girl presented with ulcerations on the right thigh for one month, and painful swelling of the right leg and right buttock for six months. The right lower limb and vulva were swollen, tender and hard with hyperpigmentation and inguinal lymphadenopathy. She had severe anaemia, eosinophilia and negative serology for HIV I and II. Histopathological examination showed a dermal chronic granulomatous infiltrate with fungal hyphae and yeast forms suggestive of Basidiobolus ranarum. There was marked reduction in right leg size and inguinal lymphadenopathy after several weeks of oral itraconazole, and complete healing of ulcers after 10 weeks. The purpose of this report is to increase awareness of this disfiguring condition which is treatable but, if not correctly diagnosed, could result in inappropriate interventions such as amputation and anti-coagulant therapy. FUNDING: None declared.

Colonic basidiobolomycosis with liver involvement masquerading as gastrointestinal lymphoma: a case report and literature review

Abstract Basidiobolomycosis is an unusual fungal skin infection that rarely involves the gastrointestinal tract. This study reported a 5-year-old boy with gastrointestinal basidiobolomycosis that had been misdiagnosed as gastrointestinal lymphoma. He was treated by surgical resection and a combination of posaconazole and amphotericin B deoxycholate with an acceptable response and no recurrence.

Experimental infection in gerbils by Conidiobolus lamprauges.

Conidiobolomycosis is an emerging entomophthoramycosis caused by fungi Conidiobolus spp. Animal models are essential for the study of infectious disease in various areas such as pathogenesis, diagnostic methods, treatment and prevention. There is not currently an animal model for conidiobolomycosis. The aim of this study was to create an experimental infection protocol for Conidiobolus lamprauges in gerbils (Meriones unguiculatus). The study animals were randomly divided into four groups of four animals: immunosuppressed with cyclophosphamide (CPA) and infected with C. lamprauges (G1), immunocompetent and infected with C. lamprauges (G2), immunosuppressed with CPA (G3), and an immunocompetent control group (G4). Clinical signs were observed only in G1 animals, where the mortality rate reached 75% by day 7 after infection (AI) with a median survival of 2 days. C. lamprauges was detected only in G1, both by PCR and by isolation. Necropsies of the G1 animals showed lesions in the nasal cavity and lung tissue. These lesions were characterized by polymorphonuclear infiltrate cells and by the presence of hyphal structures under silver staining. This animal model will be useful for further investigation of diseases caused by C. lamprauges, particularly of those associated with immunosuppression factors in naturally occurring animal infections.

[A case of rhinofacial entomophthoromycosis in Soudano-Sahelian tropical climate in Burkina Faso]. / Un cas d'entomophthoromycose rhinofaciale en climat tropical soudano-sahélien au Burkina Faso.

We describe a rhinofacial entomophthoramycosis case in a sexagenarian (65 years old) housewife. She was immunocompetent and resident of Burkina Faso. She consulted both the service of dermatology and the service of stomatology of the Teaching Hospital of Bobo-Dioulasso in February 2016 for a diffuse facial tumefaction evolving over six months. This tumefaction was associated with headaches and a left nasal obstruction. Histological examination of the lesion showed an important and polymorphic inflammatory reaction. Also, a filamentous fungus with wide non-septated hyphae and right-angled fungal branching, consistent with mucormycosis was isolated. Mycological diagnosis based on fungal culture with Sabouraud medium without any antibiotic and cyclohexemide after incubation at 27°C and at 30°C was negative. Furthermore, it was not possible to amplify the DNA extracted from biopsy. Antifungal therapy based on the administration of fluconazole per os at 800mg/day was started allowing clinical improvement. This is the first case of a rhinofacial entomophtharomycosis documented in Bobo-Dioulasso. Rhinofacial entomophthoromycosis is largely unknown, even in tropical regions such as Burkina Faso. This lack of knowledge results in a delay in the diagnosis, and subsequently a bad prognosis. It is therefore urgent to improve knowledge on this disease to guide diagnostic steps, prognosis of outcome, and antifungal therapy.