Introduction
Background
Necrotizing fasciitis (NF) is an insidiously advancing soft tissue infection characterized by widespread fascial necrosis. A number of bacteria in isolation or as a polymicrobial infection can cause necrotizing fasciitis.1 The organisms most closely linked to necrotizing fasciitis are group A beta-hemolytic streptococci, although the disease may also be caused by other bacteria or different streptococcal serotypes.
Necrotizing fasciitis was first described in 1848. In 1920, Meleney identified 20 patients in China in whom hemolytic streptococcus was the sole organism. Wilson coined the term necrotizing fasciitis in 1952 and found no specific pathologic bacteria related to the disease.
A few distinct necrotizing fasciitis syndromes should be recognized. The 3 most important are type I, or polymicrobial; type II, or group A streptococcal; and type III gas gangrene, or clostridial myonecrosis. A variant of necrotizing fasciitis type I is saltwater necrotizing fasciitis, in which an apparently minor skin wound is contaminated with saltwater containing a Vibrio species.
Necrotizing fasciitis may occur as a complication of a variety of surgical procedures or medical conditions, including cardiac catheterization,2 veinsclerotherapy,3 and diagnostic laparoscopy,4 among others5,6,7,8,9,10 . Familiarity with necrotizing fasciitis may facilitate earlier diagnosis and initiation of appropriate therapy.
Images of necrotizing fasciitis are included below.
Left upper extremity shows necrotizing fascitis in an individual who used illicit drugs. Cultures grew Streptococcus milleri and anaerobes (Prevotella species). Patient would grease, or lick, the needle before injection.
Left lower extremity in a 56-year-old patient with alcoholism who was found comatose after binge drinking. Surgical drainage was performed to treat the pyomyositis-related, large, non–foul-smelling (sweetish) bullae. Gram staining showed the presence of gram-positive rods. Cultures revealed Clostridium perfringens. The diagnosis was clostridial myonecrosis.
Sixty-year-old woman who had undergone postvaginal hysterectomy and repair of a rectal prolapse has a massive perineal ulceration with foul-smelling discharge. Cultures revealed Escherichia coli and Bacteroides fragilis. The diagnosis was peroneal gangrene.
Necrotizing fascitis at a possible site of insulin injection in the left upper part of the thigh in a 50-year-old obese woman with diabetes.
Necrotizing fascitis of entire thoracolumbar posterior area in 20-year-old patient with chronic myelogenous leukemia and neutropenia (WBC count, 680/microL). Cultures revealed gram-negative Pseudomonas species and Bacteroides fragilis.
A related eMedicine article with an emergency medicine focus is Necrotizing Fasciitis.
Pathophysiology
Organisms spread from the subcutaneous tissue along the superficial and deep fascial planes, presumably facilitated by bacterial enzymes and toxins. This deep infection causes vascular occlusion, ischemia, and tissue necrosis. Superficial nerves are damaged, producing the characteristic localized anesthesia. Septicemia ensues with systemic toxicity.
Important bacterial factors include surface protein expression and toxin production. M-1 and M-3 surface proteins, which increase the adherence of the streptococci to the tissues, also protect the bacteria against phagocytosis by neutrophils.
Streptococcal pyrogenic exotoxins (SPEs) A, B, and C are directly toxic and tend to be produced by strains causing necrotizing fasciitis. These pyrogenic exotoxins, together with streptococcal superantigen (SSA), lead to the release of cytokines and produce clinical signs such as hypotension. The etiological agent may also be a Staphylococcus aureus isolate harboring the enterotoxin gene cluster seg, sei, sem, sen, and seo but lacking all common toxin genes, including Panton-Valentine leukocidin.11 The poor prognosis associated with necrotizing fasciitis has been linked to infection with certain streptococcal strains. Community-acquired methicillin-resistant S aureus has also been associated with necrotizing fasciitis.12
Severe myositis accompanying septic necrotizing fasciitis may be caused by a Panton-Valentine leukocidinpositive S aureus strain.14 Immunostaining may document strong binding of the Panton-Valentine leukocidin toxin to necrotic muscle tissues.
Mortality/Morbidity
The mortality rate for necrotizing fasciitis can be as high as 25%. Cases of necrotizing fasciitis with sepsis and renal failure have a mortality rate as high as 70%.
Age
Approximately half of the cases of streptococcal necrotizing fasciitis occur in young and previously healthy people.
Clinical
History
Necrotizing fasciitis tends to begin with constitutional symptoms of fever and chills. After 2-3 days, erythema is noted, and supralesional vesiculation or bullae formation ensues. Serosanguineous fluid may drain from the affected area. Necrotizing fasciitis may develop after skin biopsy; at needle puncture sites in those use illicit drugs; and after episodes of frostbite, chronic venous leg ulcers, open bone fractures, insect bites, surgical wounds, and skin abscesses. However, in many cases, no association with such factors can be made. Necrotizing fasciitis may also occur in the setting of diabetes mellitus, surgery, trauma, or infectious processes.
In a pediatric necrotizing fasciitis series, clinical features began 1 week after the initiating event, beginning with edema and induration, which was followed in 24-48 hours by erythema or a violaceous discoloration.15 Pain and, occasionally, crepitation, was also noted early. The latter indicates the presence of gas produced by aerobic and anaerobic bacteria and is highly suggestive of the diagnosis of necrotizing fasciitis. In a series of 39 pediatric cases, the most common initiating factor in 13 of them was varicella.16
- Type I, or polymicrobial necrotizing fasciitis, usually occurs after trauma or surgery.
- This form may initially be mistaken for a simple wound cellulitis. However, severe pain and systemic toxicity reflect widespread tissue necrosis underlying apparently viable skin.
- This disease process may also be observed in association with urogenital or anogenital infections (see Physical, below).17
- Type II, or group A streptococcal necrotizing fasciitis, is the so-called flesh-eating bacterial infection.7
- Type III necrotizing fasciitis, or clostridial myonecrosis, is gas gangrene. This skeletal muscle infection may be associated with recent surgery or trauma.
Physical
- General findings in necrotizing fasciitis may include the following:
- From a rapidly advancing erythema, painless ulcers may appear as the infection spreads along the fascial planes. A black necrotic eschar may be evident at the borders of the affected areas.
- Metastatic cutaneous plaques may occur.
- Septicemia is typical and leads to severe systemic toxicity and rapid death unless appropriately treated.
- In individuals with diabetes, crepitus is often evident, as are nonclostridial anaerobic infections.
- Purpura with or without bullae formation, occasionally with a lack of cutaneous erythema and heat, may be found, which does not preclude the diagnosis of necrotizing fasciitis.18
- In type II necrotizing fasciitis, the widespread tissue necrosis underlying the apparently viable skin can be demonstrated by passing a probe through the tissue.
- The condition may appear similar to a simple wound cellulitis; however, the severe pain and systemic toxicity reflect the widespread tissue necrosis underlying the apparently viable skin. Surgically confirmed necrotizing fasciitis cases were compared with 12 patients with superficial soft tissue infection.19 Necrotizing fasciitis patients were more likely to have skin areas of ischemia or necrosis, fluid-filled vesicles, and severe sepsis or septic shock.
- Gas may be evident.
- This process may also be observed in the perineum in association with urogenital or anogenital infections (eg, Fournier gangrene).
- In type II necrotizing fasciitis, gas usually is not evident in affected tissues.
- Although the following features can occur with cellulitis, they may instead suggest necrotizing fasciitis:
- Rapid progression
- Poor therapeutic response
- Blistering necrosis
- Cyanosis
- Extreme local tenderness
- High temperature
- Tachycardia
- Hypotension
- Altered level of consciousness
Causes
- Group A beta-hemolytic streptococci is not the only cause of necrotizing fasciitis. Haemophilus aphrophilus and S aureus are also associated with the condition, and some patients have mixed infections involving multiple species of bacteria, including mycobacteria, or fungi.20,21
- A synergistic infection with a facultative anaerobic bacterium may be significant.
- In 1 patient, Phycomycetes appeared to be responsible for necrotizing fasciitis.
- Diabetes mellitus and immunosuppression predispose patients to necrotizing fasciitis.
- However, approximately one half of the cases of streptococcal necrotizing fasciitis occur in young and previously healthy people.
- Streptococcus pneumoniae is a rare cause of necrotizing fasciitis.22 In one patient, S pneumoniae serotype 5 was also isolated. This serotype 5 antigen is included in the polysaccharide 23-valent pneumococcal vaccine, highlighting the value of pneumococcal immunization.
- In type I necrotizing fasciitis, anaerobic and facultative bacteria work synergistically to cause what may initially be mistaken for a simple wound cellulitis. A variant of type I necrotizing fasciitis is saltwater necrotizing fasciitis in which an apparently minor skin wound is contaminated with saltwater containing a Vibrio species.
- In type II necrotizing fasciitis, varicella infection and the use of nonsteroidal anti-inflammatory drugs may be predisposing factors.
- Type III necrotizing fasciitis is usually caused by Clostridium perfringens. When type III necrotizing fasciitis occurs spontaneously, Clostridium septicum is more likely to be the etiologic agent; these cases usually occur in association with colon cancer or leukemia.
- Rapidly progressive necrotizing fasciitis following a stonefish sting has been described in 2 patients.23
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References
Kihiczak GG, Schwartz RA, Kapila R. Necrotizing fasciitis: a deadly infection. J Eur Acad Dermatol Venereol. Apr 2006;20(4):365-9. [Medline].
Federman DG, Kravetz JD, Kirsner RS. Necrotizing fasciitis and cardiac catheterization. Cutis. Jan 2004;73(1):49-52. [Medline].
Chan HT, Low J, Wilson L, Harris OC, Cheng AC, Athan E. Case cluster of necrotizing fasciitis and cellulitis associated with vein sclerotherapy. Emerg Infect Dis. Jan 2008;14(1):180-1. [Medline].
Bharathan R, Hanson M. Diagnostic laparoscopy complicated by group A streptococcal necrotizing fasciitis. J Minim Invasive Gynecol. Jan-Feb 2010;17(1):121-3. [Medline].
Akcay EK, Cagil N, Yulek F, et al. Necrotizing fasciitis of eyelid secondary to parotitis. Eur J Ophthalmol. Jan-Feb 2008;18(1):128-30. [Medline].
Anwar UM, Ahmad M, Sharpe DT. Necrotizing fasciitis after liposculpture. Aesthetic Plast Surg. Nov-Dec 2004;28(6):426-7. [Medline].
Bisno AL, Cockerill FR 3rd, Bermudez CT. The initial outpatient-physician encounter in group A streptococcal necrotizing fasciitis. Clin Infect Dis. Aug 2000;31(2):607-8. [Medline].
Gibbon KL, Bewley AP. Acquired streptococcal necrotizing fasciitis following excision of malignant melanoma. Br J Dermatol. Oct 1999;141(4):717-9. [Medline].
Sewell GS, Hsu VP, Jones SR. Zoster gangrenosum: necrotizing fasciitis as a complication of herpes zoster. Am J Med. Apr 15 2000;108(6):520-1. [Medline].
Tung-Yiu W, Jehn-Shyun H, Ching-Hung C, Hung-An C. Cervical necrotizing fasciitis of odontogenic origin: a report of 11 cases. J Oral Maxillofac Surg. Dec 2000;58(12):1347-52; discussion 1353. [Medline].
Morgan WR, Caldwell MD, Brady JM, Stemper ME, Reed KD, Shukla SK. Necrotizing fasciitis due to a methicillin-sensitive Staphylococcus aureus isolate harboring an enterotoxin gene cluster. J Clin Microbiol. Feb 2007;45(2):668-71. [Medline].
Cheng NC, Chang SC, Kuo YS, Wang JL, Tang YB. Necrotizing fasciitis caused by methicillin-resistant Staphylococcus aureus resulting in death. A report of three cases. J Bone Joint Surg Am. May 2006;88(5):1107-10. [Medline].
Olsen RJ, Sitkiewicz I, Ayeras AA, et al. Decreased necrotizing fasciitis capacity caused by a single nucleotide mutation that alters a multiple gene virulence axis. Proc Natl Acad Sci U S A. Jan 12 2010;107(2):888-93. [Medline].
Lehman D, Tseng CW, Eells S, Miller LG, Fan X, Beenhouwer DO, et al. Staphylococcus aureus Panton-Valentine leukocidin targets muscle tissues in a child with myositis and necrotizing fasciitis. Clin Infect Dis. Jan 1 2010;50(1):69-72. [Medline].
Fustes-Morales A, Gutierrez-Castrellon P, Duran-Mckinster C, Orozco-Covarrubias L, Tamayo-Sanchez L, Ruiz-Maldonado R. Necrotizing fasciitis: report of 39 pediatric cases. Arch Dermatol. Jul 2002;138(7):893-9. [Medline].
Ford LM, Waksman J. Necrotizing fasciitis during primary varicella. Pediatrics. Jun 2000;105(6):1372-3; author reply 1373-5. [Medline].
Dahm P, Roland FH, Vaslef SN, et al. Outcome analysis in patients with primary necrotizing fasciitis of the male genitalia. Urology. Jul 2000;56(1):31-5; discussion 35-6. [Medline].
Iwata Y, Sato S, Murase Y, et al. Five cases of necrotizing fasciitis: lack of skin inflammatory signs as a clinical clue for the fulminant type. J Dermatol. Nov 2008;35(11):719-25. [Medline].
Zahar JR, Goveia J, Lesprit P, Brun-Buisson C. Severe soft tissue infections of the extremities in patients admitted to an intensive care unit. Clin Microbiol Infect. Jan 2005;11(1):79-82. [Medline].
Tang WM, Ho PL, Yau WP, Wong JW, Yip DK. Report of 2 fatal cases of adult necrotizing fasciitis and toxic shock syndrome caused by Streptococcus agalactiae. Clin Infect Dis. Oct 2000;31(4):E15-7. [Medline].
Sendi P, Johansson L, Dahesh S, et al. Bacterial phenotype variants in group B streptococcal toxic shock syndrome. Emerg Infect Dis. Feb 2009;15(2):223-32. [Medline].
Tang WM, Ho PL, Yau WP, Wong JW, Yip DK. Report of 2 fatal cases of adult necrotizing fasciitis and toxic shock syndrome caused by Streptococcus agalactiae. Clin Infect Dis. Oct 2000;31(4):E15-7. [Medline].
Tang WM, Fung KK, Cheng VC, Lucke L. Rapidly progressive necrotising fasciitis following a stonefish sting: a report of two cases. J Orthop Surg (Hong Kong). Apr 2006;14(1):67-70. [Medline].
Bahebeck J, Sobgui E, Fonfoe L, Nonga BN, Mbanya JC, Sosso M. Limb-threatening and Life-threatening Diabetic Extremities: Clinical Patterns and Outcomes in 56 Patients. J Foot Ankle Surg. January - February 2010;49(1):43-46. [Medline].
Simonart T, Simonart JM, Derdelinckx I, et al. Value of standard laboratory tests for the early recognition of group A beta-hemolytic streptococcal necrotizing fasciitis. Clin Infect Dis. Jan 2001;32(1):E9-12. [Medline].
Arslan A, Pierre-Jerome C, Borthne A. Necrotizing fasciitis: unreliable MRI findings in the preoperative diagnosis. Eur J Radiol. Dec 2000;36(3):139-43. [Medline].
Ramirez-Schrempp D, Dorfman DH, Baker WE, Liteplo AS. Ultrasound soft-tissue applications in the pediatric emergency department: to drain or not to drain?. Pediatr Emerg Care. Jan 2009;25(1):44-8. [Medline].
Bakleh M, Wold LE, Mandrekar JN, Harmsen WS, Dimashkieh HH, Baddour LM. Correlation of histopathologic findings with clinical outcome in necrotizing fasciitis. Clin Infect Dis. Feb 1 2005;40(3):410-4. [Medline].
Lota AS, Altaf F, Shetty R, Courtney S, McKenna P, Iyer S. A case of necrotising fasciitis caused by Pseudomonas aeruginosa. J Bone Joint Surg Br. Feb 2010;92(2):284-5. [Medline].
Korhonen K. Hyperbaric oxygen therapy in acute necrotizing infections with a special reference to the effects on tissue gas tensions. Ann Chir Gynaecol Suppl. 2000;7-36. [Medline].
Korhonen K, Kuttila K, Niinikoski J. Tissue gas tensions in patients with necrotising fasciitis and healthy controls during treatment with hyperbaric oxygen: a clinical study. Eur J Surg. Jul 2000;166(7):530-4. [Medline].
Krenk L, Nielsen HU, Christensen ME. Necrotizing fasciitis in the head and neck region: an analysis of standard treatment effectiveness. Eur Arch Otorhinolaryngol. Aug 2007;264(8):917-22. [Medline].
Hsiao CT, Weng HH, Yuan YD, Chen CT, Chen IC. Predictors of mortality in patients with necrotizing fasciitis. Am J Emerg Med. Feb 2008;26(2):170-5. [Medline].
Light TD, Choi KC, Thomsen TA, et al. Long-term outcomes of patients with necrotizing fasciitis. J Burn Care Res. Jan-Feb 2010;31(1):93-9. [Medline].
Afifi RY, El-Hindawi AA. Acute necrotizing fasciitis in Egyptian patients. Int J Surg. Jan 28 2008;[Medline].
Brandt MM, Corpron CA, Wahl WL. Necrotizing soft tissue infections: a surgical disease. Am Surg. Oct 2000;66(10):967-70; discussion 970-1. [Medline].
Cox NH. Streptococcal necrotizing fasciitis and the dermatologist. Br J Dermatol. Oct 1999;141(4):613-4. [Medline].
Elliott D, Kufera JA, Myers RA. The microbiology of necrotizing soft tissue infections. Am J Surg. May 2000;179(5):361-6. [Medline].
Hassell M, Fagan P, Carson P, Currie BJ. Streptococcal necrotising fasciitis from diverse strains of Streptococcus pyogenes in tropical northern Australia: case series and comparison with the literature. BMC Infect Dis. Dec 16 2004;4(1):60. [Medline].
Hsieh T, Samson LM, Jabbour M, Osmond MH. Necrotizing fasciitis in children in eastern Ontario: a case-control study. CMAJ. Aug 22 2000;163(4):393-6. [Medline].
Urschel JD. Necrotizing soft tissue infections. Postgrad Med J. Nov 1999;75(889):645-9. [Medline].
Wall DB, de Virgilio C, Black S, Klein SR. Objective criteria may assist in distinguishing necrotizing fasciitis from nonnecrotizing soft tissue infection. Am J Surg. Jan 2000;179(1):17-21. [Medline].
Wall DB, Klein SR, Black S, de Virgilio C. A simple model to help distinguish necrotizing fasciitis from nonnecrotizing soft tissue infection. J Am Coll Surg. Sep 2000;191(3):227-31. [Medline].
Further Reading
Keywords
necrotizing fasciitis, hospital gangrene, acute infective gangrene, necrotizing erysipelas, hemolytic streptococcus gangrene, suppurative fasciitis, flesh-eating bacterial infection, killer bug disease, Fournier's gangrene, Fournier gangrene, polymicrobial necrotizing fasciitis, group A streptococcal necrotizing fasciitis, gas gangrene, clostridial myonecrosis, fascial necrosis, group A beta-hemolytic streptococci, septicemia
streptococcal pyrogenic exotoxins, streptococcal superantigen, frostbite, chronic venous leg ulcers, open bone fractures, insect bites, surgical wounds, skin abscesses, diabetes mellitus, violaceous discoloration, black necrotic eschar, metastatic cutaneous plaques, nonclostridial anaerobic infections, blistering necrosis, cyanosis, Haemophilus aphrophilus, Staphylococcus, phycomycetes
Vibrio species, varicella infection, Clostridium perfringens, Clostridium septicum, colon cancer, leukemia










Overview: Necrotizing Fasciitis