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Disease Profile

Necrotizing fasciitis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Categories

Bacterial infections

Summary

Necrotizing fasciitis is a serious infection of the skin, the tissue just beneath the skin (subcutaneous tissue), and the tissue that covers internal organs (fascia). Necrotizing fasciitis can be caused by several different types of bacteria, and the infection can arise suddenly and spread quickly. Early signs include flu-like symptoms and redness and pain around the infection site. A prompt diagnosis and treatment are essential. If the infection is not treated promptly, it can lead to multiple organ failure and death. Treatment typically includes intravenous (IV) antibiotics and surgery to remove infected and dead tissue.[1][2][3]

Symptoms

Symptoms often begin within hours of an injury and typically include intense pain and tenderness over the affected area. The pain is often severe and may resemble that of a torn muscle. Early symptoms may be mistaken for the flu and can include fever, sore throat, stomach ache, nausea, diarrhea, chills, and general body aches. The patient may notice redness around the area that spreads quickly; the affected area can eventually become swollen, shiny, discolored, and hot to the touch. In addition, ulcers or blisters may develop. If the infection continues to spread, the patient may experience dehydration, high fever, fast heart rate, and low blood pressure. Pain may actually improve as tissues and the nerves are destroyed. As the infection spreads, vital organs may be affected and the patient may become confused or delirious. If not successfully treated, necrotizing fasciitis can lead to shock and eventual death. [1][2][3][4]

Cause

Anyone can develop necrotizing fasciitis.[2] The most common cause is group A Streptococcus. Other types of bacteria that can cause necrotizing fasciitis include Klebsiella, Clostridium, and Escherichia coli.[3] Approximately one-half of necrotizing fasciitis cases caused by streptococcal bacteria occur in young and otherwise healthy individuals.[5] Although necrotizing fasciitis most frequently develops after trauma that causes a break in the skin, it can also develop after minor trauma that occurs without a break in the skin.[5] Necrotizing fasciitis can occur as a complication of surgery; it can also occur at the site of a relatively minor injury such as an insect bite or an injection. In addition, underlying illnesses that weaken the immune system may increase the risk of necrotizing fasciitis.[2][3][5] Some studies suggest a possible relationship between the use of nonsteroidal anti-inflammatory medications (NSAIDs) during varicella infections and the development of necrotizing fasciitis.[5] 

Treatment

Accurate and prompt diagnosis, treatment with intravenous (IV) antibiotics, and surgery to remove dead tissue are vital in treating necrotizing fasciitis. As the blood supply to the infected tissue becomes impaired, antibiotics often cannot penetrate the infected tissue. Therefore, surgery to remove the dead, damaged, or infected tissue is the primary treatment for necrotizing fasciitis.[2][3] Early surgery may minimize tissue loss, eliminating the need for amputation of the infected body part.[4] The choice of antibiotics will likely depend on the particular bacteria involved. In addition, supplemental oxygen, fluids, and medicines may be needed to raise the blood pressure.[1] Hyperbaric oxygen therapy and IV immunoglobulin may also be considered, but their use in patients with necrotizing fasciitis is controversial.[2][1]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States.
      • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
      • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Necrotizing fasciitis. Click on the link to view a sample search on this topic.

          References

          1. Necrotizing fasciitis. DermNet New Zealand Trust. March 4, 2016; https://www.dermnetnz.org/bacterial/necrotising-fasciitis.html. Accessed 4/12/2016.
          2. Sarani, Babak. Necrotizing Fasciitis. National Organization of Rare Disorders (NORD). 2015; https://rarediseases.org/rare-diseases/necrotizing-fasciitis/. Accessed 4/12/2016.
          3. Necrotizing Fasciitis: A Rare Disease, Especially for the Healthy. Centers for Disease Control and Prevention (CDC). April 17, 2015; https://www.cdc.gov/features/necrotizingfasciitis/. Accessed 4/12/2016.
          4. Edlich, Richard. Necrotizing Fasciitis. Medscape Reference. July 9, 2015; https://emedicine.medscape.com/article/2051157-overview. Accessed 4/13/2016.
          5. Edlich, Richard. Necrotizing Fasciitis. Medscape Reference. July 9, 2015; https://emedicine.medscape.com/article/2051157-overview. Accessed 4/12/2016.

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