II. Definitions

  1. Necrotizing Fasciitis
    1. Rapidly progressive, deep subcutaneous infection
  2. Fournier's Gangrene
    1. Massive infection and swelling of Scrotum and penis
    2. Extends into perineum or abdominal wall, and legs

III. Pathophysiology

  1. Infection spreads between fascia and SQ tissue
  2. Fibrous bands prevent infectious spread
    1. Present in head and distal extremities
    2. Lacking in trunk and proximal extremities

IV. Risk factors

  1. Age over 50 years
  2. Malnutrition
    1. Hypoalbuminemia
    2. Alcoholism
    3. Cirrhosis (esp. Vibrio vulnificans from seafood)
  3. Immunocompromised state
    1. Cancer
    2. Corticosteroid use
  4. Poor vascular supply
    1. Cardiovascular disease
    2. Peripheral Vascular Disease
    3. Diabetes Mellitus
  5. Skin Trauma within last 90 days
    1. Burn Injury
    2. Trauma
    3. Intravenous Drug Abuse
    4. Recent surgery
    5. Alcohol Abuse
  6. Miscellaneous risk factors
    1. Obesity
  7. Break in Gastrointestinal or Genitourinary mucosa
    1. Colon Cancer
    2. Diverticula
    3. Hemorrhoids or Anal Fissure
    4. Urethral tear
    5. Cirrhosis

V. Findings: Symptoms and Signs progression (in order of occurrence)

  1. Pain
  2. Unexplained fever
  3. Swelling
  4. Brawny Edema and tenderness
  5. Dark red induration
  6. Bullae filled with blue or purple fluid
  7. Skin friable, bluish, maroon, or black
  8. Extensive thrombosis of dermal blood vessels
  9. Extension to deep fascia leads to brown-gray appearance
  10. Rapid spread along fascial planes, veins and lymph
  11. Toxicity, shock, and multi-organ failure

VI. Signs: Distribution

  1. Extremities (53%)
  2. Perineum or buttocks (20%, especially in Diabetes Mellitus, Alcoholism)
  3. Trunk (18%)
  4. Head and neck (9%)
  5. References
    1. Bosshardt (1996) Arch Surg 131:846-52 [PubMed]

VII. Etiologies

  1. Polymicrobial - Mixed aerobic and Anaerobic Bacteria
    1. Break in Gastrointestinal or Genitourinary mucosa, typically on trunk and extremities
    2. Fournier's Gangrene
    3. Comorbid conditions associated with mixed infection
      1. Diabetes Mellitus
      2. Peripheral Vascular Disease
      3. Immunocompromised state
  2. Group A Streptococcus (Streptococcus Pyogenes) with or without Staphylococcus aureus
    1. Begins deep at non-penetrating minor Trauma in typically healthy patients
    2. Risk Factors include IV Drug Abuse and skin popping
    3. Contusion seeded by transient bacteremia
    4. Gas production only if mixed infection
    5. Severe toxicity, renal Impairment may precede shock
    6. Myositis in 20-40% cases
      1. Creatine Phosphokinase (CPK) is markedly elevated
    7. Mortality: 20-50% despite Penicillin
  3. Other Bacterial causes
    1. Staphylococcus aureus
    2. Clostridium perfringens
      1. Hyperbaric Oxygen treatment may help in Gas Gangrene
    3. Vibrio haemlyticus or Vibrio vulnificus (sea water exposure)
    4. Aeromonas (fresh water exposure)
    5. Plesiomonas (fresh water exposure)
    6. Superinfection of varicella in children
    7. Omphalitis (Umbilical Cord stump infection) in newborns

VIII. Diagnosis: Findings Suggestive of Necrotizing Fasciitis

  1. Altered Mental Status
  2. Soft tissue erythema, edema and severe pain (70-80% of cases)
    1. Pain out of proportion to the exam
    2. Tenderness outside the erythematous borders
  3. Vessicles or Bullae (25%)
    1. Bullae become violaceous after 4-5 days
    2. Skin then becomes necrotic
    3. Hemorrhagic bullae are nearly pathognomonic for Necrotizing Fasciitis (esp. Vibrio vulnificus)
  4. Fever (40%)
    1. Temperature over 99.5 F (37.5 C)
  5. Hypotension (21%)
    1. Systolic Blood Pressure <90 mmHg
  6. Crepitation (20%)

IX. Diagnosis: Definitive

  1. Deep tissie biposy with culture and Gram Stain

X. Labs

  1. See Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC Score)
  2. Complete Blood Count
    1. White Blood Cell Count over 15,000/mm3 (esp. if >25,000/mm3)
    2. Hemoglobin less than 11 g/dl
    3. Platelet Count <150,000 per mm3
  3. Serum Electrolytes
    1. Serum Sodium under 135 meq/L (SIADH related)
    2. Blood Urea Nitrogen 15 mg/dl
    3. Serum Calcium under 8.4 mg/dl
    4. Serum Creatinine >1.6 mg/dl
  4. Coagulation Studies
    1. Prothrombin Time (PT) prolonged
    2. Partial Thromboplastin Time (aPTT) prolonged
  5. Other markers
    1. C-Reactive Protein >150 mg/L
    2. Arterial or venous pH < 7.35

XI. Imaging

  1. XRay
    1. Test Sensitivity: 25% for subcutaneous gas
    2. Gas along fascial planes is pathognomonic for Necrotizing Fasciitis
  2. MRI
    1. Efficacy
      1. Test Sensitivity: 100%
      2. Test Specificity: 86%
    2. Findings
      1. Deep intermuscular fascia involvement
      2. Fascial thickening (>3mm)
      3. Thickened fascia completely lacks signal enhancement post-Gadolinium
    3. References
      1. Malghem (2013) Joint Bone Spine 80(2): 146-54 [PubMed]

XIII. Management: Surgical exploration to fascia and muscle

  1. Early exploration and surgical debridement within 12 hours is critical (delay risks higher mortality)
  2. Observe for
    1. Necrotizing Fasciitis
    2. Myositis
    3. Gangrene
  3. Technique
    1. Visualize deep structures
    2. Remove necrotic materials
    3. Reduce compartment pressure
    4. Send material for Gram Stain and Culture

XIV. Management: Empiric

  1. Combination Regimen (3 drugs plus MRSA coverage)
    1. Anaerobe coverage (and inhibits ribosomal production of toxins)
      1. Clindamycin 600 mg IV every 8 hours
    2. Gram Positive coverage
      1. Ampicillin-sulbactam (Unasyn) 1.5 to 3 g IV every 6-8 hours OR
      2. Piperacillin-tazobactam 3.375 IV q6-8 hours
    3. Gram Negative coverage
      1. Ciprofloxacin 400 mg IV every 12 hours
  2. Combination Regimen (2 drugs plus MRSA)
    1. Cefotaxime 2 grams IV every 6 hours AND
    2. Anaerobic coverage
      1. Metronidazole 50 mg IV every 6 hours OR
      2. Clindamycin 600 mg IV every 8 hours
  3. Single agent regimens (choose one plus MRSA coverage)
    1. Imipenem-Cilastin 1 g IV every 6-8 hours
    2. Meropenem 1 g IV every 8 hours
    3. Ertapenem 1 g IV every 24 hours
  4. MRSA Coverage (use with above regimens)
    1. Vancomycin 15 mg/kg IV every 12 hours
    2. Linezolid 600 mg IV every 12 hours
    3. Clindamycin not typically recommended for MRSA as of 2015 due to growing resistance
      1. Review local antibiograms
  5. Other antibiotics
    1. Add Doxycycline if hemorrhagic bullae are seen (cover for Vibrio vulnificus)
  6. Other measures
    1. Maximize nutritional status

XV. Management: Post-exposure Prophylaxis

  1. Indications
    1. Household contact exposure from onset to 48 hours from antibiotic start
  2. Treatment options (choose one)
    1. Penicillin G Benzathine
      1. Weight <60 pounds (<27 kg): 600,000 units IM for 1 dose or
      2. Weight >60 pounds (<>7 kg): 1,200,000 units IM for 1 dose
    2. Rifampin 5 mg/kg/day (up to 300 mg maximum) orally twice daily for 4 days
    3. Clindamycin 20 mg/kg (up to 300 mg maximum) orally three times daily for 10 days
    4. Azithromycin (Zithromax) 12 mg/kg (up to 500 mg) orally daily for 5 days
  3. References
    1. Sablier (2010) Lancet 375(9719): 1052 [PubMed]

XVI. Prognosis

  1. Mortality: 25-35% (up to 70% in those who develop Sepsis, 100% with delayed diagnosis, management)

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Ontology: Necrotizing fasciitis (C0238124)

Definition (NCI) Infection of the deep skin and subcutaneous tissues and necrosis of the fascia. It is caused by bacteria including group A streptococcus, Staphylococcus aureus and Clostridium perfringens. It may develop following trauma and invasive procedures.
Definition (MSH) A fulminating bacterial infection of the deep layers of the skin and FASCIA. It can be caused by many different organisms, with STREPTOCOCCUS PYOGENES being the most common.
Concepts Disease or Syndrome (T047)
MSH D019115
ICD9 728.86
ICD10 M72.6
SnomedCT 52486002, 186428007, 320701000009109
English Fasciitides, Necrotizing, Fasciitis, Necrotizing, Fascitides, Necrotizing, Fascitis, Necrotizing, Necrotizing Fasciitides, Necrotizing Fascitides, Necrotizing Fascitis, Streptococcal gangrene, Necrotizing fasciitis NOS, FASCIITIS NECROTISING, FASCIITIS NECROTIZING, necrotizing fasciitis, necrotizing fasciitis (diagnosis), Fasciitis necrotising, Necrotising fasciitis NOS, Fasciitis necrotizing, Necrotizing Fasciitis, Fasciitis, Necrotizing [Disease/Finding], necrotizing cellulitis, fasciitis necrotize, fascitis necrotizing, necrotizing myositis, streptococcal gangrene, necrotising fasciitis, fasciitis necrotising, fasciitis necrotizing, necrotizing fascitis, Necrotizing erysipelas, Necrotising erysipelas, Necrotising fasciitis (disorder), Necrotizing myositis, Necrotising cellulitis, Necrotising fasciitis, Necrotizing cellulitis, Necrotising myositis, Necrotizing fasciitis (disorder), Necrotizing fasciitis
Dutch necrotiserende fasciïtis NAO, necrotiserende cellulitis, fasciïtis necrotiserend, necrotiserende fasciïtis, Fasciitis necroticans, Necrotiserende fasciitis
French Cellulite nécrosante, Fasciite nécrosante SAI, FASCIITE NECROSANTE, Fasciite nécrosante, Cellulite gangréneuse, Fasciite gangréneuse, Gangrène sous-cutanée à streptocoques bêta-hémolytiques, Syndrome de Meleney
German Fasziitis nekrotisierend, nekrotisierende Zellulitis, nekrotisierende Entzuendung der Faszien NNB, nekrotisierende Fasziitis, Fasziitis, nekrotisierende
Italian Fascite necrotizzante NAS, Cellulite necrotizzante, Fascite necrotizzante
Portuguese Celulite necrosante, Fasceíte necrosante, Fasceíte necrosante NE, FASCEITE NECROSANTE, Fasciite necrotizante, Fasciite Necrosante
Spanish Fascitis necrotizante, Celulitis necrotizante, Fasciitis necrosante, Fasciitis necrotizante NEOM, Fascitis necrosante, miosistis necrotizante, erisipela necrotizante, erisipela necrosante, fascitis necrosante (trastorno), fascitis necrosante, fascitis necrotizante, miositis necrosante, Fasciitis necrotizante, Fascitis Necrotizante
Russian НЕКРОТИЧЕСКАЯ ФОРМА РОЖИ, NEKROTICHESKAIA FORMA ROZHI, ROZHI NEKROTICHESKAIA FORMA, FASTSIT NEKROTICHESKII, РОЖИ НЕКРОТИЧЕСКАЯ ФОРМА, ФАСЦИТ НЕКРОТИЧЕСКИЙ
Japanese 壊死性筋膜炎NOS, 壊死性蜂巣炎, エシセイキンマクエン, エシセイホウソウエン, エシセイキンマクエンNOS, 筋膜炎-壊死性, 壊死性筋膜炎
Swedish Fascit, nekrotiserande
Czech fasciitida nekrotizující, Nekrotizující celulitida, Nekrotizující fasciitida, Nekrotizující fasciitida NOS
Finnish Nekrotisoiva faskiitti
Korean 괴사 근막염
Polish Zapalenie powięzi martwicze
Hungarian necrotizáló fasciitis, cellulitis necrotisans, Necrotizáló fasciitis k.m.n., Fasciitis necrotisans, Necrotizáló cellulitis, Nekrotizáló fasciitis
Norwegian Nekrotiserende fasciitt, Fasciitt, nekrotiserende

Ontology: Fournier Gangrene (C0238419)

Definition (MSHCZE) Infekce skrota a hráze pokračující do gangrény, vyvolaná smíšenou bakteriální flórou a kandidou. Zejm. u diabetiků. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) An acute necrotic infection of the SCROTUM; PENIS; or PERINEUM. It is characterized by scrotum pain and redness with rapid progression to gangrene and sloughing of tissue. Fournier gangrene is usually secondary to perirectal or periurethral infections associated with local trauma, operative procedures, or urinary tract disease.
Concepts Disease or Syndrome (T047)
MSH D018934
ICD10 N49.3
SnomedCT 398318005, 236742003
English Fournier Disease, Fournier Gangrene, Fournier's Disease, Fournier's Gangrene, Fourniers Disease, Fourniers Gangrene, Gangrene, Fournier's, FOURNIERS DIS, FOURNIER DIS, Gangrene, Fournier, Fournier gangrene, Fournier Gangrene [Disease/Finding], fournier disease, fournier gangrene, fournier's gangrene, fourniers gangrene, fourniers disease, gangrene fournier's, fournier's disease, fournier gangrene (diagnosis), Fournier's gangrene, Fournier's gangrene (disorder), Fournier; gangrene, gangrene; Fournier
German Fournier-Gangraen, Fournier-Krankheit, Fournier-Gangrän, Skrotalgangrän
Japanese フルニエー壊疽, フルニエーエソ, Fournier壊疽, Fournier病, フルニエ壊疽, フルニエ病
Swedish Fourniers gangrän
Czech Fournierova nemoc, Fournierova gangréna
Finnish Fournierin gangreeni
Russian GANGRENA FURN'E, FURN'E BOLEZN', FURN'E GANGRENA, ГАНГРЕНА ФУРНЬЕ, ФУРНЬЕ БОЛЕЗНЬ, ФУРНЬЕ ГАНГРЕНА
Italian Malattia di Fournier, Gangrena di Fournier
Croatian FOURNIEROVA GANGRENA, GANGRENA FOURNIER
Polish Choroba Fourniera, Zgorzel Fourniera, Zgorzel moszny
Hungarian Fournier-gangraena
Norwegian Fourniers sykdom, Fourniers gangren
Dutch Fournier; gangreen, gangreen; Fournier, Fournier-gangreen, Fournier-ziekte, Gangreen, Fournier-
Spanish gangrena de Fournier (trastorno), gangrena de Fournier, Gangrena de Fournier, Enfermedad de Fournier
French Gangrène de Fournier, Gangrène génitale, Gangrène périnéo-scrotale, Gangrène périnéoscrotale, Maladie de Fournier
Portuguese Doença de Fournier, Gangrena de Fournier

Ontology: Streptococcal necrotizing fasciitis (C0854084)

Concepts Disease or Syndrome (T047)
SnomedCT 449900006
Italian Fascite necrotizzante streptococcica
French Fasciite nécrosante à streptocoques, Fasciite nécrosante streptococcique
Spanish Fascitis necrosante por estreptococos, fascitis necrosante debida a Streptococcus pyogenes, fascitis necrosante debida a Streptococcus pyogenes (trastorno), gangrena estreptocócica, Fasciitis necrosante estreptocócica
Japanese レンサ球菌性壊死性筋膜炎, レンサキュウキンセイエシセイキンマクエン
Czech Streptokoková nekrotizující fasciitida
English Necrotising fasciitis streptococcal, Necrotizing fasciitis streptococcal, Necrotizing fasciitis due to Streptococcus pyogenes, Type II necrotising fasciitis, Streptococcal gangrene, Streptococcal necrotising fasciitis, Necrotizing fasciitis due to Streptococcus pyogenes (disorder), Type II necrotizing fasciitis, Necrotising fasciitis due to Streptococcus pyogenes, Streptococcal necrotizing fasciitis, necrotizing fasciitis due to streptococcus pyogenes (diagnosis)
Hungarian streptococcalis necrotizáló fasciitis, Necrotizáló streptococcalis fasciitis
Portuguese Fasceíte necrosante estreptocócica
Dutch necrotiserende streptokokkenfasciïtis
German nekrotisierende Fasziitis durch Streptokokken