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Potentially life-threatening emergency
Mucormycosis in Child
See also in: External and Internal Eye,Pulmonary
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Potentially life-threatening emergency

Mucormycosis in Child

See also in: External and Internal Eye,Pulmonary
Print Images (6)
Contributors: Vivian Wong MD, PhD, Susan Burgin MD, Harvey A. Brown MD, William Bonnez MD
Other Resources UpToDate PubMed

Synopsis

Mucormycosis is infection caused by genera of fungi in the order Mucorales (most commonly Rhizopus, Mucor, and Rhizomucor fungi; less commonly Absidia, Cunninghamella, Saksenaea, and Apophysomyces). The agents of mucormycosis are ubiquitously distributed in decaying matter, including bread, vegetables, fruits, and seeds. They grow and sporulate abundantly, and clinical infection is usually acquired by inhalation of the spores. Inoculation of the spores through abraded skin can lead to primary cutaneous infection.

Mucormycosis can cause serious life-threatening infection in compromised hosts. While rhinocerebral mucormycosis is the most common form of the disease, mucormycosis can involve the skin, pulmonary, renal, and gastrointestinal organs, or it can disseminate. The underlying risk factor can influence the clinical manifestation; rhinocerebral disease is common in people with diabetes, whereas patients with hematological malignancies or neutropenia usually have pulmonary disease and, more rarely, gastrointestinal disease. Renal transplant patients are more likely to have involvement of the graft kidney.

The 5 major types of mucormycosis are:
  • Rhino-orbital-cerebral disease – Patients present with unilateral facial pain, headaches, fever, nasal or sinus congestion and discharge, and black, necrotic ulcerations usually involving the palate. Infection can erode locally and spread to the cavernous sinus, the internal carotid artery, and the brain. Orbital involvement can lead to orbital cellulitis, ophthalmoplegia, and multiple cranial nerve palsies, with a potential for permanent residual effects such as blindness and cranial nerve defects occurring up to 70% of the time. These patients may present with only a central retinal artery occlusion.
  • Pulmonary disease – This typically manifests as cough, dyspnea, and unremitting fever despite broad-spectrum antibiotics. Hemoptysis occurs as a result of tissue necrosis and angio-invasion and, rarely, can be fatal. Physical examination findings are nonspecific and include tachypnea, crackles, decreased breath sounds, and wheezing.
  • Cutaneous disease – This form of mucormycosis may be primary or secondary. Primary infection may be associated with direct inoculation from trauma, burns, and surgical wounds. The affected tissue becomes inflamed, followed by subsequent necrosis. Dissemination is rare. Secondary cutaneous mucormycosis occurs from hematogenous spread.
  • Gastrointestinal disease – This form is mainly seen in extreme cases of malnutrition or immunosuppression. It is believed to occur via direct ingestion of spores. Clinical findings may include abdominal pain, nausea, vomiting, gastrointestinal bleeding, fever, and change in bowel habits. It is the most common form of mucormycosis in newborns.
  • Disseminated disease – This most commonly occurs following pulmonary mucormycosis, although it could be a result of any subtype. The brain is the most susceptible to infection, followed by the spleen, heart, skin, and other organs.
In addition to uncontrolled diabetes mellitus and diabetic ketoacidosis, hematological malignancies, and neutropenia, major risk factors for invasive disease include lymphomas, deferoxamine therapy, malnourishment, and immunosuppression as a result of solid organ and stem cell transplantation. Corticosteroid therapy has been cited as a predisposing factor. Other patients at risk for invasive disease are surgical patients, burn patients, trauma patients, injection drug users, and newborn infants.

In patients with human immunodeficiency virus (HIV), mucormycosis is rare, but when it does occur, it can do so without the typical predisposing factors. In patients with HIV / AIDS, mucormycosis tends to occur in intravenous drug abusers, patients with prolonged and severe neutropenia, and individuals with very low CD4 counts. Systemic toxicity can be severe and progress rapidly, or the disease can progress insidiously. Death frequently occurs. Mucormycosis may complicate previous bacterial infection.

In immunocompetent hosts, epidemic mucormycosis has been associated with penetrating and nonpenetrating trauma caused by natural disasters, eg, the tornado in Joplin, MO, in 2011. DNA analysis in that case showed an Apophysomyces trapeziformis species.

For discussion of diseases caused by fungi belonging to the order of Entomophthorales, see Cutaneous basidiobolomycosis, Disseminated basidiobolomycosis, and Rhinofacial conidiobolomycosis.

Codes

ICD10CM:
B46.3 – Cutaneous mucormycosis

SNOMEDCT:
76627001 – Mucormycosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Ecthyma gangrenosum in patients who are immunocompromised.
  • The clinical manifestations of mucormycosis are similar to that of other invasive fungi like Aspergillus, Fusarium, and Pseudallescheria spp. Mucor appears as broad, non-septate hyphae with branches occurring at right angles; rarely, septa may be seen. The other fungi have thinner, septate hyphae with frequent, acute-angled branching.
  • Leishmaniasis (Old World and New World)
  • Tularemia
  • Candida sepsis
Orbital infection: Gastrointestinal mucormycosis may be misdiagnosed as necrotizing enterocolitis in a newborn.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 11/05/2018
Last Updated: 11/05/2018
Copyright © 2018 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Mucormycosis in Child
See also in: External and Internal Eye,Pulmonary
Print 6 Images
View all Images (6)
(with subscription)
Mucormycosis (Cutaneous) : Fever, Bite or trauma site, Skin ulcer, Painful skin lesion, Black eschar
Clinical image of Mucormycosis
Copyright © 2018 VisualDx®. All rights reserved.