Necrotizing fasciitis
Encyclopedia
Necrotizing fasciitis commonly known as flesh-eating disease or Flesh-eating bacteria syndrome, is a rare
infection
of the deeper layers of skin
and subcutaneous tissues, easily spreading across the fascia
l plane within the subcutaneous tissue.
Necrotizing fasciitis is a quickly progressing and severe disease of sudden onset and is usually treated immediately with high doses of intravenous antibiotics.
Type I describes a polymicrobial infection, whereas Type II describes a monomicrobial infection. Many types of bacteria can cause necrotizing fasciitis (e.g., Group A streptococcus (Streptococcus pyogenes)
, Staphylococcus aureus
, Vibrio vulnificus
, Clostridium perfringens
, Bacteroides fragilis
). Such infections are more likely to occur in people with compromised immune systems.
Historically, Group A streptococcus made up most cases of Type II infections. However, since as early as 2001, another serious form of monomicrobial necrotizing fasciitis has been observed with increasing frequency. In these cases, the bacterium causing it is methicillin-resistant Staphylococcus aureus (MRSA)
, a strain of S. aureus that is resistant
to methicillin
, the antibiotic
used in the laboratory that determines the bacterium's sensitivity to flucloxacillin
or nafcillin that would be used for treatment clinically.
Several studies have demonstrated a link between absorption of non-steroidal anti-inflammatory drugs and flesh-eating disease, though it has not been established whether the drugs just masked the symptoms or were a cause per se.
The infection begins locally at a site of trauma
, which may be severe (such as the result of surgery
), minor, or even non-apparent. Patients usually complain of intense pain that may seem excessive given the external appearance of the skin. With progression of the disease, often within hours, tissue becomes swollen. Diarrhea and vomiting are also common symptoms.
In the early stages, signs of inflammation may not be apparent if the bacteria are deep within the tissue. If they are not deep, signs of inflammation, such as redness and swollen or hot skin, develop very quickly. Skin color may progress to violet, and blisters may form, with subsequent necrosis
(death) of the subcutaneous tissue
s.
Patients with necrotizing fasciitis typically have a fever
and appear very ill. Mortality rates have been noted as high as 73 percent if left untreated. Without surgery and medical assistance, such as antibiotic
s, the infection will rapidly progress and will eventually lead to death.
, as the bacteria do not actually "eat" the tissue. They cause the destruction of skin and muscle by releasing toxin
s (virulence factors), which include streptococcal pyogenic exotoxins. S. pyogenes produces an exotoxin known as a superantigen
. This toxin is capable of activating T-cells non-specifically, which causes the overproduction of cytokines and severe systemic illness (Toxic shock syndrome
).
Patients are typically taken to surgery based on a high index of suspicion, determined by the patient's signs and symptoms. In necrotizing fasciitis, aggressive surgical debridement
(removal of infected tissue) is always necessary to keep it from spreading and is the only treatment available. Diagnosis is confirmed by visual examination of the tissues and by tissue samples sent for microscopic evaluation.
Early medical treatment is often presumptive; thus, antibiotics should be started as soon as this condition is suspected. Initial treatment often includes a combination of intravenous antibiotics including penicillin
, vancomycin
, and clindamycin
. Cultures are taken to determine appropriate antibiotic coverage, and antibiotics may be changed when culture results are obtained.
As in other maladies characterized by massive wounds or tissue destruction, hyperbaric oxygen treatment can be a valuable adjunctive therapy but is not widely available. Amputation
of the affected organ(s) may be necessary. Repeat explorations usually need to be done to remove additional necrotic tissue. Typically, this leaves a large open wound, which often requires skin grafting
. The associated systemic inflammatory response is usually profound, and most patients will require monitoring in an intensive care unit
.
Treatment for necrotizing fasciitis may involve an interdisciplinary care team. For example, in the case of a necrotizing fasciitis involving the head and neck, the team could include otolaryngologists, intensivists, microbiologists and plastic surgeons.
Rare disease
A rare disease, also referred to as an orphan disease, is any disease that affects a small percentage of the population.Most rare diseases are genetic, and thus are present throughout the person's entire life, even if symptoms do not immediately appear...
infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...
of the deeper layers of skin
Skin
-Dermis:The dermis is the layer of skin beneath the epidermis that consists of connective tissue and cushions the body from stress and strain. The dermis is tightly connected to the epidermis by a basement membrane. It also harbors many Mechanoreceptors that provide the sense of touch and heat...
and subcutaneous tissues, easily spreading across the fascia
Fascia
A fascia is a layer of fibrous tissue that permeates the human body. A fascia is a connective tissue that surrounds muscles, groups of muscles, blood vessels, and nerves, binding those structures together in much the same manner as plastic wrap can be used to hold the contents of sandwiches...
l plane within the subcutaneous tissue.
Necrotizing fasciitis is a quickly progressing and severe disease of sudden onset and is usually treated immediately with high doses of intravenous antibiotics.
Type I describes a polymicrobial infection, whereas Type II describes a monomicrobial infection. Many types of bacteria can cause necrotizing fasciitis (e.g., Group A streptococcus (Streptococcus pyogenes)
Group A streptococcal infection
The group A streptococcus bacterium is a form of β-hemolytic Streptococcus bacteria responsible for most cases of streptococcal illness. Other types may also cause infection...
, Staphylococcus aureus
Staphylococcus aureus
Staphylococcus aureus is a facultative anaerobic Gram-positive coccal bacterium. It is frequently found as part of the normal skin flora on the skin and nasal passages. It is estimated that 20% of the human population are long-term carriers of S. aureus. S. aureus is the most common species of...
, Vibrio vulnificus
Vibrio vulnificus
Vibrio vulnificus is a species of Gram-negative, motile, curved, rod-shaped bacteria of the Vibrio Genus. It was first reported by Hollis et al. in 1976. It was subsequently given the name Beneckea vulnifica by Reichelt et al. in 1976 , and finally Vibrio vulnificus by Farmer in 1979...
, Clostridium perfringens
Clostridium perfringens
Clostridium perfringens is a Gram-positive, rod-shaped, anaerobic, spore-forming bacterium of the genus Clostridium. C. perfringens is ever present in nature and can be found as a normal component of decaying vegetation, marine sediment, the intestinal tract of humans and other vertebrates,...
, Bacteroides fragilis
Bacteroides fragilis
Bacteroides fragilis is a Gram-negative bacillus bacterium species, and an obligate anaerobe of the gut.B. fragilis group is the most commonly isolated bacteriodaceae in anaerobic infections especially those that originate from the gastrointestinal flora. B. fragilis is the most prevalent organism...
). Such infections are more likely to occur in people with compromised immune systems.
Historically, Group A streptococcus made up most cases of Type II infections. However, since as early as 2001, another serious form of monomicrobial necrotizing fasciitis has been observed with increasing frequency. In these cases, the bacterium causing it is methicillin-resistant Staphylococcus aureus (MRSA)
Methicillin-resistant Staphylococcus aureus
Methicillin-resistant Staphylococcus aureus is a bacterium responsible for several difficult-to-treat infections in humans. It is also called multidrug-resistant Staphylococcus aureus and oxacillin-resistant Staphylococcus aureus...
, a strain of S. aureus that is resistant
Antibiotic resistance
Antibiotic resistance is a type of drug resistance where a microorganism is able to survive exposure to an antibiotic. While a spontaneous or induced genetic mutation in bacteria may confer resistance to antimicrobial drugs, genes that confer resistance can be transferred between bacteria in a...
to methicillin
Methicillin
Meticillin or methicillin is a narrow-spectrum beta-lactam antibiotic of the penicillin class. It should not be confused with the antibiotic metacycline.-History:Methicillin was developed by Beecham in 1959...
, the antibiotic
Antibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
used in the laboratory that determines the bacterium's sensitivity to flucloxacillin
Flucloxacillin
Flucloxacillin or floxacillin is a narrow-spectrum beta-lactam antibiotic of the penicillin class. It is used to treat infections caused by susceptible Gram-positive bacteria. Unlike other penicillins, flucloxacillin has activity against beta-lactamase-producing organisms such as Staphylococcus...
or nafcillin that would be used for treatment clinically.
Several studies have demonstrated a link between absorption of non-steroidal anti-inflammatory drugs and flesh-eating disease, though it has not been established whether the drugs just masked the symptoms or were a cause per se.
Signs and symptoms
Over 70% of cases are recorded in patients with one of the following clinical situations: immunosuppression, diabetes, alcoholism/drug abuse, malignancies, and chronic systemic diseases. It occasionally occurs in people with an apparently normal general condition.The infection begins locally at a site of trauma
Trauma
Trauma can refer to:-In psychology and medicine:* Trauma , an often serious and body-altering physical injury, such as the removal of a limb...
, which may be severe (such as the result of surgery
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
), minor, or even non-apparent. Patients usually complain of intense pain that may seem excessive given the external appearance of the skin. With progression of the disease, often within hours, tissue becomes swollen. Diarrhea and vomiting are also common symptoms.
In the early stages, signs of inflammation may not be apparent if the bacteria are deep within the tissue. If they are not deep, signs of inflammation, such as redness and swollen or hot skin, develop very quickly. Skin color may progress to violet, and blisters may form, with subsequent necrosis
Necrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
(death) of the subcutaneous tissue
Subcutaneous tissue
The hypodermis, also called the hypoderm, subcutaneous tissue, or superficial fascia is the lowermost layer of the integumentary system in vertebrates. Types of cells that are found in the hypodermis are fibroblasts, adipose cells, and macrophages...
s.
Patients with necrotizing fasciitis typically have a fever
Fever
Fever is a common medical sign characterized by an elevation of temperature above the normal range of due to an increase in the body temperature regulatory set-point. This increase in set-point triggers increased muscle tone and shivering.As a person's temperature increases, there is, in...
and appear very ill. Mortality rates have been noted as high as 73 percent if left untreated. Without surgery and medical assistance, such as antibiotic
Antibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
s, the infection will rapidly progress and will eventually lead to death.
Pathophysiology
"Flesh-eating bacteria" is a misnomerMisnomer
A misnomer is a term which suggests an interpretation that is known to be untrue. Such incorrect terms sometimes derive their names because of the form, action, or origin of the subject becoming named popularly or widely referenced—long before their true natures were known.- Sources of misnomers...
, as the bacteria do not actually "eat" the tissue. They cause the destruction of skin and muscle by releasing toxin
Toxin
A toxin is a poisonous substance produced within living cells or organisms; man-made substances created by artificial processes are thus excluded...
s (virulence factors), which include streptococcal pyogenic exotoxins. S. pyogenes produces an exotoxin known as a superantigen
Superantigen
Superantigens are a class of antigens which cause non-specific activation of T-cells resulting in oligoclonal T cell activation and massive cytokine release...
. This toxin is capable of activating T-cells non-specifically, which causes the overproduction of cytokines and severe systemic illness (Toxic shock syndrome
Toxic shock syndrome
Toxic shock syndrome is a potentially fatal illness caused by a bacterial toxin. Different bacterial toxins may cause toxic shock syndrome, depending on the situation. The causative bacteria include Staphylococcus aureus and Streptococcus pyogenes...
).
LRINEC score
The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score can be utilized to risk stratify patients presenting with signs of cellulitis to determine the likelihood of necrotizing fasciitis being present. It uses six serologic measures: C-reactive protein, total white cell count, hemoglobin, sodium, creatinine and glucose. A score greater than 6 indicates that necrotizing fasciitis should be seriously considered. The scoring criteria are as follows- CRP (mg/L) >150 - 4 points
- WBC count (per mm3)
- <15 - 0 points
- 15-25 - 1 point
- >25 - 2 points
- Hemoglobin (g/dL)
- >13.5 - 0 points
- 11-13.5 - 1 point
- <11 - 2 points
- Sodium (mmol/L) <135 - 2 points
- Creatinine (umol/L) >141 - 2 points
- Glucose (mmol/L) >10 - 1 point
Treatment
Debridement
Debridement is the medical removal of a patient's dead, damaged, or infected tissue to improve the healing potential of the remaining healthy tissue...
(removal of infected tissue) is always necessary to keep it from spreading and is the only treatment available. Diagnosis is confirmed by visual examination of the tissues and by tissue samples sent for microscopic evaluation.
Early medical treatment is often presumptive; thus, antibiotics should be started as soon as this condition is suspected. Initial treatment often includes a combination of intravenous antibiotics including penicillin
Penicillin
Penicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
, vancomycin
Vancomycin
Vancomycin INN is a glycopeptide antibiotic used in the prophylaxis and treatment of infections caused by Gram-positive bacteria. It has traditionally been reserved as a drug of "last resort", used only after treatment with other antibiotics had failed, although the emergence of...
, and clindamycin
Clindamycin
Clindamycin rINN is a lincosamide antibiotic. It is usually used to treat infections with anaerobic bacteria but can also be used to treat some protozoal diseases, such as malaria...
. Cultures are taken to determine appropriate antibiotic coverage, and antibiotics may be changed when culture results are obtained.
As in other maladies characterized by massive wounds or tissue destruction, hyperbaric oxygen treatment can be a valuable adjunctive therapy but is not widely available. Amputation
Amputation
Amputation is the removal of a body extremity by trauma, prolonged constriction, or surgery. As a surgical measure, it is used to control pain or a disease process in the affected limb, such as malignancy or gangrene. In some cases, it is carried out on individuals as a preventative surgery for...
of the affected organ(s) may be necessary. Repeat explorations usually need to be done to remove additional necrotic tissue. Typically, this leaves a large open wound, which often requires skin grafting
Skin grafting
Skin grafting is a type of graft surgery involving the transplantation of skin. The transplanted tissue is called a skin graft.Skin grafting is often used to treat:*Extensive wounding or trauma*Burns...
. The associated systemic inflammatory response is usually profound, and most patients will require monitoring in an intensive care unit
Intensive Care Unit
thumb|220px|ICU roomAn intensive-care unit , critical-care unit , intensive-therapy unit/intensive-treatment unit is a specialized department in a hospital that provides intensive-care medicine...
.
Treatment for necrotizing fasciitis may involve an interdisciplinary care team. For example, in the case of a necrotizing fasciitis involving the head and neck, the team could include otolaryngologists, intensivists, microbiologists and plastic surgeons.
Notable people afflicted
- King Herod the Great of Judea may have suffered from Fournier gangreneFournier gangreneFournier's gangrene is a type of necrotizing infection or gangrene usually affecting the perineum.It was first described by Baurienne in 1764 and is named after a French venereologist, Jean-Alfred Fournier following five cases he presented in clinical lectures in 1883.-Cause:In the majority of...
(necrotizing fasciitis of the groin and genitalia) at the time of his death, as suggested in a "historical autopsy." - Lucien BouchardLucien BouchardLucien Bouchard, is a Canadian lawyer, diplomat, politician and former Minister of the Environment of the Canadian Federal Government. He was the Leader of Opposition in the Canadian House of Commons from 1993 to 1996, and the 27th Premier of Quebec from January 29, 1996 to March 8, 2001...
, former premier of QuébecQuebecQuebec or is a province in east-central Canada. It is the only Canadian province with a predominantly French-speaking population and the only one whose sole official language is French at the provincial level....
, Canada, who became infected in 1994 while leader of the federal official opposition Bloc QuébécoisBloc QuébécoisThe Bloc Québécois is a federal political party in Canada devoted to the protection of Quebec's interests in the House of Commons of Canada, and the promotion of Quebec sovereignty. The Bloc was originally a party made of Quebec nationalists who defected from the federal Progressive Conservative...
party, lost a leg to the illness. - Melvin FranklinMelvin FranklinDavid Melvin English better known by the stage name Melvin Franklin, was an American bass singer. Franklin is best known for his role as a member of Motown singing group The Temptations from 1960 to 1994....
, bass singer for The TemptationsThe TemptationsThe Temptations is an American vocal group having achieved fame as one of the most successful acts to record for Motown Records. The group's repertoire has included, at various times during its five-decade career, R&B, doo-wop, funk, disco, soul, and adult contemporary music.Formed in Detroit,...
. Though Franklin's condition was diagnosed early enough to prevent complete amputation of his arm, he died from other health complications soon afterward in 1995. - Jeff MooradJeff MooradJeff Moorad is a former sports agent and is currently lead owner of the San Diego Padres and part-owner of the Arizona Diamondbacks baseball team.He is an Assyrian American...
, former agent and partial owner of the San Diego PadresSan Diego PadresThe San Diego Padres are a Major League Baseball team based in San Diego, California. They play in the National League Western Division. Founded in 1969, the Padres have won the National League Pennant twice, in 1984 and 1998, losing in the World Series both times...
and Arizona DiamondbacksArizona DiamondbacksThe Arizona Diamondbacks are a professional baseball team based in Phoenix. They play in the West Division of Major League Baseball's National League. From 1998 to the present, they have played in Chase Field...
, contracted the disease in 1997. He had seven surgeries in a little more than a week but later recovered fully. - Eric Allin CornellEric Allin CornellEric Allin Cornell is an American physicist who, along with Carl E. Wieman, was able to synthesize the first Bose–Einstein condensate in 1995...
, winner of the 2001 Nobel Prize in PhysicsNobel Prize in PhysicsThe Nobel Prize in Physics is awarded once a year by the Royal Swedish Academy of Sciences. It is one of the five Nobel Prizes established by the will of Alfred Nobel in 1895 and awarded since 1901; the others are the Nobel Prize in Chemistry, Nobel Prize in Literature, Nobel Peace Prize, and...
, lost his left arm and shoulder to the disease in 2004. - Jan Peter BalkenendeJan Peter BalkenendeJan Pieter "Jan Peter" Balkenende is a Dutch politician of the party Christian Democratic Appeal .He was the Prime Minister of the Netherlands from 22 July 2002 until 14 October 2010, having led four coalition governments, cabinets Balkenende I, II, III and IV, none of which served a full...
, former Prime Minister of the NetherlandsPrime Minister of the NetherlandsThe Prime Minister of the Netherlands is the chairman of the Council of Ministers of the Netherlands. He is the de facto head of government of the Netherlands and coordinates the policy of the government...
was infected in 2004. He was in the hospital for several weeks but recovered fully. - Alexandru MarinAlexandru MarinAlexandru Adalbert "Alex" Marin was an experimental particle physicist, a professor of physics at MIT, Boston University and Harvard University, and a researcher at CERN and JINR....
, an experimental particle physicist, professor at MITMassachusetts Institute of TechnologyThe Massachusetts Institute of Technology is a private research university located in Cambridge, Massachusetts. MIT has five schools and one college, containing a total of 32 academic departments, with a strong emphasis on scientific and technological education and research.Founded in 1861 in...
, Boston UniversityBoston UniversityBoston University is a private research university located in Boston, Massachusetts. With more than 4,000 faculty members and more than 31,000 students, Boston University is one of the largest private universities in the United States and one of Boston's largest employers...
and Harvard UniversityHarvard UniversityHarvard University is a private Ivy League university located in Cambridge, Massachusetts, United States, established in 1636 by the Massachusetts legislature. Harvard is the oldest institution of higher learning in the United States and the first corporation chartered in the country...
, and researcher at CERNCERNThe European Organization for Nuclear Research , known as CERN , is an international organization whose purpose is to operate the world's largest particle physics laboratory, which is situated in the northwest suburbs of Geneva on the Franco–Swiss border...
and JINRJoint Institute for Nuclear ResearchThe Joint Institute for Nuclear Research, JINR , in Dubna, Moscow Oblast , Russia, is an international research centre for nuclear sciences, with 5500 staff members, 1200 researchers including 1000 Ph.D.s from eighteen member states The Joint Institute for Nuclear Research, JINR , in Dubna, Moscow...
, died from the disease in 2005. - David WaltonDavid WaltonDavid Robert Walton was a British economist, and a member of the Bank of England's Monetary Policy Committee from July 2005 until his death in June 2006....
, a leading economist in the UK and a member of the Bank of England's Monetary Policy Committee, which is responsible for setting interest rates, died of the disease within 24 hours of diagnosis on June 21, 2006. - Alan CorenAlan CorenAlan Coren was an English humorist, writer and satirist who was well known as a regular panellist on the BBC radio quiz The News Quiz and a team captain on BBC television's Call My Bluff...
, British writer and satirist, announced in his Christmas 2006 column for The TimesThe TimesThe Times is a British daily national newspaper, first published in London in 1785 under the title The Daily Universal Register . The Times and its sister paper The Sunday Times are published by Times Newspapers Limited, a subsidiary since 1981 of News International...
that his long absence as a columnist had been due to contracting the disease while on holiday in France. - R. W. Johnson, South African journalist and historian, contracted the disease in March 2009 after injuring his foot while swimming. His leg was amputated above the knee.
- Jeff HannemanJeff HannemanJeffrey John "Jeff" Hanneman is a rhythm/lead guitarist and founding member of the American thrash metal band Slayer. Hanneman grew up in Los Angeles in a family of war veterans, and his fascination with warfare is attributed to his upbringing...
, guitarist for the thrash-metal band SlayerSlayerSlayer is an American thrash metal band formed in Huntington Park, California, in 1981 by guitarists Jeff Hanneman and Kerry King. Slayer rose to fame with their 1986 release, Reign in Blood, and is credited as one of the "Big Four" thrash metal acts, along with Metallica, Megadeth and...
, contracted the disease in early 2011. He was allegedly infected after being bitten by a spider. - Peter Watts, Canadian science fiction author, contracted the disease in early 2011. On his blog, Watts reported, "I’m told I was a few hours away from being dead.... If there was ever a disease fit for a science fiction writer, flesh-eating disease has got to be it. This {*@*#*!} spread across my leg as fast as a Star TrekStar TrekStar Trek is an American science fiction entertainment franchise created by Gene Roddenberry. The core of Star Trek is its six television series: The Original Series, The Animated Series, The Next Generation, Deep Space Nine, Voyager, and Enterprise...
space disease in time-lapse."
See also
- MucormycosisMucormycosisZygomycosis is the broadest term to refer to infections caused by bread mold fungi of the zygomycota phylum. However, because zygomycota has been identified as polyphyletic, and is not included in modern fungal classification systems, the diseases that Zygomycosis can refer to are better called by...
, a rare fungal infection that can present like necrotizing fasciitis - Toxic shock syndromeToxic shock syndromeToxic shock syndrome is a potentially fatal illness caused by a bacterial toxin. Different bacterial toxins may cause toxic shock syndrome, depending on the situation. The causative bacteria include Staphylococcus aureus and Streptococcus pyogenes...
- CellulitisCellulitisCellulitis is a diffuse inflammation of connective tissue with severe inflammation of dermal and subcutaneous layers of the skin. Cellulitis can be caused by normal skin flora or by exogenous bacteria, and often occurs where the skin has previously been broken: cracks in the skin, cuts, blisters,...
- Fournier gangreneFournier gangreneFournier's gangrene is a type of necrotizing infection or gangrene usually affecting the perineum.It was first described by Baurienne in 1764 and is named after a French venereologist, Jean-Alfred Fournier following five cases he presented in clinical lectures in 1883.-Cause:In the majority of...