RARE DISEASE
Zygomycosis
Zygomycosis
Zygomycosis
Synonyms: Mucormycosis
Synonyms: Mucormycosis
Synonyms: Mucormycosis
Drug discovery
6
drugs
With orphan designations
Overview
Zygomycosis (Mucormycosis) is a life-threatening angioinvasive fungal infection caused by Mucorales molds, primarily affecting immunocompromised individuals such as those with diabetes mellitus, hematologic malignancies, or transplant recipients. Common presentations include rhino-orbital-cerebral, pulmonary, and cutaneous forms, characterized by rapid tissue necrosis. Diagnosis relies on histopathology and imaging, with aggressive treatment combining antifungals (liposomal amphotericin B) and surgical debridement [1][5][15][17].
Population
High-risk groups: Uncontrolled diabetes (especially ketoacidosis), hematologic malignancies, solid organ/stem cell transplants, long-term corticosteroid use, and COVID-19 patients with secondary immunosuppression [1][7][12][17].
Higher prevalence in India (~0.14/1,000 population) compared to Western countries (0.7–1.2/million) [7][17].
Burden
Mortality: 44%–96% depending on site (disseminated >90%, rhinocerebral ~60%) [9][14][17].
U.S. estimates: 1,140 hospitalizations (2019), 232 deaths (2021); global COVID-19-associated surges (e.g., 45,000+ cases in India, 2021) [4][7][14][17].
Healthcare costs: Direct medical expenses exceed $129M annually in the U.S., with prolonged hospital stays [4][14].
Therapies
First-line: Liposomal amphotericin B (5–10 mg/kg/day) ± early surgical debridement [3][8][13].
Salvage/adjunctive: Posaconazole/isavuconazole, iron chelation (deferasirox), and immunomodulators (e.g., GM-CSF) [3][8][17].
Surgical intervention: Critical for localized disease to remove necrotic tissue [9][15].
Categories: rare infectious diseases
Research Papers
2,185 drug discovery papers about Zygomycosis, with 29 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
2,185 drug discovery papers about Zygomycosis, with 29 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
2026-08-12 | "Adjunctive topical amphotericin B in post-transplant surgical-site mucormycosis in an immunocompromised diabetic patient: a case report".
Cutaneous mucormycosis is a rapidly progressive and often fatal fungal infection predominantly affecting immunocompromised hosts, with post-transplant patients being particularly vulnerable due to immunosuppressive regimens and metabolic derangements. Despite its high mortality, especially in the context of surgical-site involvement, optimal therapeutic strategies remain underexplored. We present a rare case of surgical-site mucormycosis in a renal transplant recipient with poorly controlled diabetes, successfully managed using adjunctive topical antifungal therapy, highlighting a potential treatment strategy in immunosuppressed populations. A middle-aged male with chronic kidney disease, recent renal transplantation, and persistent hyperglycemia developed a non-healing surgical-site wound. Early clinical suspicion led to a bedside potassium hydroxide mount and expedited fungal cultures, which revealed mucormycosis. The patient was promptly initiated on systemic liposomal amphotericin B and, uniquely, adjunctive topical amphotericin B applied directly to the wound bed. Despite the challenges of ongoing immunosuppression, renal dysfunction, and glycemic instability, the patient demonstrated significant local and systemic improvement without the need for disfiguring surgical debridement. This case underscores the diagnostic value of bedside fungal microscopy in resource-limited and time-sensitive settings, where conventional culture and histopathology may be delayed. The clinical decision to incorporate topical amphotericin B, an underutilized yet mechanistically sound adjunct, was based on the rationale of high local fungal burden, systemic drug toxicity concerns, and limited evidence in immunosuppressed surgical cohorts. This approach not only curtailed disease progression but also preserved graft integrity and patient quality of life. This case highlights the under-recognition of mucormycosis in post-transplant surgical wounds and the absence of standardized topical antifungal protocols, representing a critical gap in transplant infectious disease management. This case report highlights that early bedside diagnosis, combined with individualized adjunctive topical amphotericin B, may help control post-transplant surgical-site mucormycosis, reduce the need for morbid surgical interventions, and warrants further evaluation for incorporation into future treatment guidelines for high-risk immunocompromised patients.
2026-08-12 | Three consecutive cases of rhinocerebral mucormycosis in calves caused by Lichtheimia ramosa infection on a beef cattle farm.
Three Japanese Black calves died within a two-month period on a beef cattle farm. Necropsy consistently revealed liquefactive necrosis of the ventral brain (extending from the olfactory bulb to the midbrain) and thymic atrophy. Histopathologically, necrosuppurative encephalitis characterized by abundant fungal hyphae was observed in all three cases. Fungal lesions also involved the olfactory mucosa, olfactory nerves, and olfactory bulb. Fungi isolated from the cerebrum of all three calves were identified as Lichtheimia ramosa. These cases were diagnosed as rhinocerebral mucormycosis, originating in the olfactory mucosa and extending to the cerebrum. Although mucormycosis is typically sporadic, this report documents the first occurrence of consecutive cases of bovine rhinocerebral mucormycosis caused by L. ramosa.
2026-08-11 | Three-dimensional printed Patient-specific Implants for Reconstruction after Post-mucormycosis Maxillofacial Defects - A Systematic Review and Meta-analysis.
Post-mucormycosis maxillary, mid-facial and mandibular defects are often extensive, multi-planar and occur in medically compromised patients, making conventional obturators, free flaps and standard implant protocols challenging. Three-dimensional (3D)-printed patient-specific implants (PSIs) have emerged as a digitally planned, titanium-based alternative, but clinical evidence remains fragmented. A systematic search of electronic databases and manual sources was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020. Studies reporting clinical outcomes of 3D-printed PSIs in patients with post-mucormycosis maxillary/mid-facial defects were included. Data on patient/defect characteristics, PSI design, complications, functional and aesthetic outcomes were extracted. Risk of bias was assessed using Joanna Briggs Institute checklists for cohort/case series and case reports. Eleven studies met the inclusion criteria. Defects were predominantly Brown Class II-III maxillectomy and complex mid-facial/orbital defects treated with computed tomography/cone-beam computed tomography-guided, additively manufactured titanium PSIs, often integrated with zygomatic anchorage. Pooled analysis of five multi-patient cohorts (n = 67) showed a PSI survival proportion of 0.96 (95% confidence interval = 0.91-1.00) at 6-24 months, with negligible heterogeneity (I 2 = 0%). Complications were mostly minor and true PSI loss was rare. Functional (mastication, speech and leakage control) and aesthetic outcomes, where reported, were generally favourable with high patient satisfaction. 3D-printed PSIs show excellent short-term survival and promising functional and aesthetic rehabilitation in extensive post-mucormycosis defects, but current evidence is limited to small, high-bias observational studies. Robust prospective and comparative research is needed before routine adoption can be recommended.
2026-08-11 | An innovative technique for facial deformity reconstruction following mucormycosis using three-dimensional printing: a case report.
Extensive destruction of the facial bones caused by mucormycosis poses a significant challenge for surgeons during the reconstruction phase. In this context, three-dimensional (3D) printing plays a pivotal role in the accurate planning and design of patient-specific implants tailored to the patient's deficient bone anatomy. A 52-year-old male patient presented with extensive destruction of the maxilla, zygomatic bones, and the floor of the nasal cavity secondary to mucormycosis. The patient was unable to phonate or swallow due to oronasal communication resulting from complete continuity between the oral and nasal cavities. Conventional reconstructive approaches typically require large volumes of autologous bone grafts, prolonged operative time, and multiple surgical stages. Accordingly, reconstruction using 3D printing technology with patient-specific titanium implants was employed. Reconstruction with a 3D-printed, patient-specific titanium implant yielded a satisfactory outcome within a single surgical procedure and a relatively short operative time. Furthermore, precise 3D planning facilitated subsequent dental rehabilitation.
2026-08-07 | Gastrointestinal Mucormycosis Masquerading as an Abdominal Mass in an Immunocompetent Child.
We report a rare case of gastrointestinal mucormycosis (GI MM) in a 5-year-old immunocompetent child presenting as an abdominal mass. Initially misdiagnosed as an inflammatory myofibroblastic tumor, the correct diagnosis was made after a surgical resection via histopathology. Prompt antifungal therapy led to recovery. This case highlights atypical presentations of GI MM and the need for vigilance in seemingly immunocompetent patients.
2026-08-12 | "Adjunctive topical amphotericin B in post-transplant surgical-site mucormycosis in an immunocompromised diabetic patient: a case report".
Cutaneous mucormycosis is a rapidly progressive and often fatal fungal infection predominantly affecting immunocompromised hosts, with post-transplant patients being particularly vulnerable due to immunosuppressive regimens and metabolic derangements. Despite its high mortality, especially in the context of surgical-site involvement, optimal therapeutic strategies remain underexplored. We present a rare case of surgical-site mucormycosis in a renal transplant recipient with poorly controlled diabetes, successfully managed using adjunctive topical antifungal therapy, highlighting a potential treatment strategy in immunosuppressed populations. A middle-aged male with chronic kidney disease, recent renal transplantation, and persistent hyperglycemia developed a non-healing surgical-site wound. Early clinical suspicion led to a bedside potassium hydroxide mount and expedited fungal cultures, which revealed mucormycosis. The patient was promptly initiated on systemic liposomal amphotericin B and, uniquely, adjunctive topical amphotericin B applied directly to the wound bed. Despite the challenges of ongoing immunosuppression, renal dysfunction, and glycemic instability, the patient demonstrated significant local and systemic improvement without the need for disfiguring surgical debridement. This case underscores the diagnostic value of bedside fungal microscopy in resource-limited and time-sensitive settings, where conventional culture and histopathology may be delayed. The clinical decision to incorporate topical amphotericin B, an underutilized yet mechanistically sound adjunct, was based on the rationale of high local fungal burden, systemic drug toxicity concerns, and limited evidence in immunosuppressed surgical cohorts. This approach not only curtailed disease progression but also preserved graft integrity and patient quality of life. This case highlights the under-recognition of mucormycosis in post-transplant surgical wounds and the absence of standardized topical antifungal protocols, representing a critical gap in transplant infectious disease management. This case report highlights that early bedside diagnosis, combined with individualized adjunctive topical amphotericin B, may help control post-transplant surgical-site mucormycosis, reduce the need for morbid surgical interventions, and warrants further evaluation for incorporation into future treatment guidelines for high-risk immunocompromised patients.
2026-08-12 | Three consecutive cases of rhinocerebral mucormycosis in calves caused by Lichtheimia ramosa infection on a beef cattle farm.
Three Japanese Black calves died within a two-month period on a beef cattle farm. Necropsy consistently revealed liquefactive necrosis of the ventral brain (extending from the olfactory bulb to the midbrain) and thymic atrophy. Histopathologically, necrosuppurative encephalitis characterized by abundant fungal hyphae was observed in all three cases. Fungal lesions also involved the olfactory mucosa, olfactory nerves, and olfactory bulb. Fungi isolated from the cerebrum of all three calves were identified as Lichtheimia ramosa. These cases were diagnosed as rhinocerebral mucormycosis, originating in the olfactory mucosa and extending to the cerebrum. Although mucormycosis is typically sporadic, this report documents the first occurrence of consecutive cases of bovine rhinocerebral mucormycosis caused by L. ramosa.
2026-08-11 | Three-dimensional printed Patient-specific Implants for Reconstruction after Post-mucormycosis Maxillofacial Defects - A Systematic Review and Meta-analysis.
Post-mucormycosis maxillary, mid-facial and mandibular defects are often extensive, multi-planar and occur in medically compromised patients, making conventional obturators, free flaps and standard implant protocols challenging. Three-dimensional (3D)-printed patient-specific implants (PSIs) have emerged as a digitally planned, titanium-based alternative, but clinical evidence remains fragmented. A systematic search of electronic databases and manual sources was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020. Studies reporting clinical outcomes of 3D-printed PSIs in patients with post-mucormycosis maxillary/mid-facial defects were included. Data on patient/defect characteristics, PSI design, complications, functional and aesthetic outcomes were extracted. Risk of bias was assessed using Joanna Briggs Institute checklists for cohort/case series and case reports. Eleven studies met the inclusion criteria. Defects were predominantly Brown Class II-III maxillectomy and complex mid-facial/orbital defects treated with computed tomography/cone-beam computed tomography-guided, additively manufactured titanium PSIs, often integrated with zygomatic anchorage. Pooled analysis of five multi-patient cohorts (n = 67) showed a PSI survival proportion of 0.96 (95% confidence interval = 0.91-1.00) at 6-24 months, with negligible heterogeneity (I 2 = 0%). Complications were mostly minor and true PSI loss was rare. Functional (mastication, speech and leakage control) and aesthetic outcomes, where reported, were generally favourable with high patient satisfaction. 3D-printed PSIs show excellent short-term survival and promising functional and aesthetic rehabilitation in extensive post-mucormycosis defects, but current evidence is limited to small, high-bias observational studies. Robust prospective and comparative research is needed before routine adoption can be recommended.
2026-08-11 | An innovative technique for facial deformity reconstruction following mucormycosis using three-dimensional printing: a case report.
Extensive destruction of the facial bones caused by mucormycosis poses a significant challenge for surgeons during the reconstruction phase. In this context, three-dimensional (3D) printing plays a pivotal role in the accurate planning and design of patient-specific implants tailored to the patient's deficient bone anatomy. A 52-year-old male patient presented with extensive destruction of the maxilla, zygomatic bones, and the floor of the nasal cavity secondary to mucormycosis. The patient was unable to phonate or swallow due to oronasal communication resulting from complete continuity between the oral and nasal cavities. Conventional reconstructive approaches typically require large volumes of autologous bone grafts, prolonged operative time, and multiple surgical stages. Accordingly, reconstruction using 3D printing technology with patient-specific titanium implants was employed. Reconstruction with a 3D-printed, patient-specific titanium implant yielded a satisfactory outcome within a single surgical procedure and a relatively short operative time. Furthermore, precise 3D planning facilitated subsequent dental rehabilitation.
2026-08-07 | Gastrointestinal Mucormycosis Masquerading as an Abdominal Mass in an Immunocompetent Child.
We report a rare case of gastrointestinal mucormycosis (GI MM) in a 5-year-old immunocompetent child presenting as an abdominal mass. Initially misdiagnosed as an inflammatory myofibroblastic tumor, the correct diagnosis was made after a surgical resection via histopathology. Prompt antifungal therapy led to recovery. This case highlights atypical presentations of GI MM and the need for vigilance in seemingly immunocompetent patients.
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Drug Discovery Landscape
6 orphan drug designations for Zygomycosis, including 2 approved therapies.
6 orphan drug designations for Zygomycosis, including 2 approved therapies.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Fosmanogepix | small molecules | EMA | 2023-02-15 | — | Basilea Pharmaceutica Deutschland GmbH |
Posaconazole | small molecules | FDA | 2021-12-13 | — | AET Pharma US, Inc. |
Isavuconazonium sulfate [Cresemba] | small molecules | EMA | 2014-06-04 | 2015-10-19 | Basilea Pharmaceutica Deutschland GmbH |
isavuconazonium sulfate [Cresemba] | small molecules | FDA | 2013-10-25 | 2015-03-06 | Astellas Pharma Global Development Inc. |
posaconazole | small molecules | FDA | 2004-07-16 | — | Merck Sharp & Dohme LLC |
Amphotericin B lipid complex | small molecules | FDA | 1996-05-06 | — | The Liposome Company, Inc. |
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