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Necrotizing Fasciitis

Necrotizing fasciitis is a rare, severe soft tissue infection typically by Group A streptococci, marked by necrosis of subcutaneous tissues with involvement of the fascia.


Presentation

During the first 2 days, the following clinical features are present:

Over the next 2 to 3 days, the clinical features include:

  • Edema
  • Formation of hemorrhagic bullae (fluid filled thin walled blisters)
  • Grey discoloration of the skin, indicating necrosis
  • Hardening of the subcutaneous tissues in the fascial planes
  • Crepitus due to production of gas in the tissues.
  • Gangrene
  • Intense pain that disappears soon due to destruction of pain nerve endings

After another 2 days, the following grave complications are seen:

Tachypnea
  • Similarly, patients may present high fever, anxiety, altered mental status, leukocytosis, shock, and tachypnea, when shock is about to develop.[frontiersin.org]
  • Tachypnea, hyperglycemia (with osmotic diuresis), and fever aggravate the hypovolemic state. 48 Tissue bacteria are isolated in about 76% of cases. 49 In our series, positive tissue cultures were found in 24 cases (62%).[jamanetwork.com]
Fever
  • These are: Fever with shock and light-headedness Fever with a sunburn rash Fever with a cough and difficulty breathing Fever with cellulitis that can progress to necrotizing fasciitis or myositis The classic warning signals are unusually severe pain at[healthcentral.com]
  • Historically, group A streptococci have been responsible for deadly outbreaks of scarlet fever , rheumatic fever , and toxic shock syndrome .[britannica.com]
  • In general, early symptoms were found to be fever, erythema, localized selling, and tenderness or pain. "Pain out of proportion" was not mentioned as a typical symptom. Fever and leukocytosis were more common in teenage patients.[ncbi.nlm.nih.gov]
  • : Group A strep (the most common) Klebsiella Clostridium E. coli Staphylococcus aureus Aeromonas hydrophila other bacterial species Symptoms Symptoms of necrotizing fasciitis include (but are not limited to): rapid onset of severe pain and swelling, fever[health.state.mn.us]
  • You can’t describe the way the pain is, and the fever I had was just unbelievable,” Madsen said. “But then, after they started the NeutroPhase and started killing all of the toxins in my arm, the fever subsided and went away.[foxnews.com]
Swelling
  • A broad differential diagnosis in case of lower lip swelling is essential to avoid inappropriate treatment delay.[ncbi.nlm.nih.gov]
  • Periocular necrotizing fasciitis developed in a 12-month-old boy with swelling of both eyes and redness and a discharge from the left eye approximately 36 hours after blunt trauma.[ncbi.nlm.nih.gov]
  • We present a 53-year-old diabetic woman who presented to the emergency room with several weeks of worsening breast and shoulder pain, swelling, and erythema.[ncbi.nlm.nih.gov]
  • A 55-year-old female patient presented with acute painful swelling and redness of the right upper eyelid that spread to both eyelids bilaterally within 24 h.[ncbi.nlm.nih.gov]
Severe Pain
  • One alerting sign is unusually severe pain - far greater than normal for a cut or wound - and painful lymph nodes.[healthcentral.com]
  • At the time of presentation, this diabetic patient was quite systemically ill (hypotensive with multi system organ failure) with severe pain at the site of infection.[meded.ucsd.edu]
  • A 50-year-old homeless African-American man presented complaining of severe pain in his right lower extremity. A clinical workup was consistent with emphysematous pyomyositis and compartment syndrome; he received limb-saving surgical intervention.[ncbi.nlm.nih.gov]
  • pain and swelling, fever, and redness at the site of the injury.[health.state.mn.us]
  • pain of her left foot.[medbullets.com]
High Fever
  • Case report A 7-year-old girl presented to Kassala New Hospital on 2 March 2005 with high fever following FGM.[emro.who.int]
  • Typically patients have high fevers and elevated white-blood-cell counts. They feel terrible and often need to go to the emergency room.[ucdenver.edu]
  • Immediate side effects include high fever, nausea, diarrhea and chills. As it progresses, the skin becomes bright red, swollen and shiny, before blistering and, in worst-case scenarios, becoming open wounds.[people.com]
  • It's not so much the chills, the high fever, nausea and stomach upset that many survivors of necrotizing fasciitis remember most. It's the pain. "[Doctors] asked me to rate the scale of pain. I said, ‘It's 100, it's 1,000.[ajc.com]
  • Patients are acutely ill, with high fever, tachycardia, altered mental status ranging from confusion to obtundation, and hypotension. Patients may be bacteremic or septic and may require aggressive hemodynamic support.[merckmanuals.com]
Anemia
  • The infection can spread up to 2.5 cm/h, with minimal change in the overlying skin. 1 Erythema, swelling, and severe pain are generally seen at presentation. 1 Bullae and soft tissue gas ( fig. ) are late signs. 1 Anemia, hyponatremia, hypoglycemia, and[anesthesiology.pubs.asahq.org]
  • ; the air is undetectable externally and may or may not be detected with x-ray CT scanning: This is more effective in detecting the gas and pinpointing the site of necrosis Complete blood count and differential: Elevated white blood cell (WBC); mild anemia[nurses.com]
  • […] infection (leukocytosis with a predominance of neutrophils), muscle damage (elevated creatine phosphokinase levels), hepatic or renal insufficiency or failure (elevated liver enzyme, blood urea nitrogen, and creatinine levels), electrolyte imbalances, and anemia[nursingcenter.com]
  • All this time the patient has been under intensive care of fluid, electrolytes and metabolic balance, received blood transfusions to redress the anemia, correction of the low albumin levels, oral and parenteral nutrition, intensive specific nursing.[tmj.ro]
  • Laboratory studies in patients with necrotizing fasciitis may reveal metabolic acidosis, leukocytosis, anemia, thrombocytopenia, coagulopathy, hyponatremia, or renal or liver dysfunction.[emdocs.net]
Trismus
  • He presented with fever, dyspnea, and inflammatory signs bilaterally in the submandibular, submental, and superior thoracic regions as well as severe trismus.[ncbi.nlm.nih.gov]
  • Clinical presentations were a rapidly progressing painful neck swelling, fever, dysphagia and trismus. The aetiology varied from idiopathic, pharyngeal/tonsillar infection, trauma and nasal malignancy.[ncbi.nlm.nih.gov]
Lip Swelling
  • A broad differential diagnosis in case of lower lip swelling is essential to avoid inappropriate treatment delay.[ncbi.nlm.nih.gov]
Tachycardia
  • Cutaneous symptoms, oliguria, metabolic acidosis, acute renal failure, severe hypotension, and tachycardia occurred, and the patient was admitted to Intensive Care a few hours after initial admission.[ncbi.nlm.nih.gov]
  • Necrotizing fasciitis is reported in half of patients with streptococcal toxic shock-like syndrome, or STSS. This illness is characterized by the presence of multiple symptoms: chills, fever or hypothermia and shock, confusion, vomiting, diarrhea, tachycardia[health.state.mn.us]
  • ASSESSMENT Fever: can be hypothermic or hyperthermic Blood pressure: may be hypotensive; tissue destruction causes fluid volume loss Heart rate: tachycardia is present, especially in the presence of fever and hypotension Pain: initially a sudden onset[nurses.com]
  • […] days, the following clinical features are present: Localized, severe, persistent pain Swelling Erythema No defined margins of the region of skin that is involved, resembling cellulitis Malaise Fever Chills and sweating Nausea and vomiting Dizziness Tachycardia[symptoma.com]
  • He initially complained of extreme thigh pain with erythema and swelling but rapidly developed bullae and worsening erythema over the affected area along with fever and tachycardia. A clinical photo is shown in Figure A.[orthobullets.com]
Blister
  • Use soap and water to clean even small breaks in your skin, such as minor cuts or blisters. Cover the wounds with a sterile bandage. Wash your hands often with soap and water.[drugs.com]
  • It’s best not to wait to treat even minor scrapes, blisters or breaks in the skin with an antibacterial ointment and clean bandages. The chance of acquiring such an aggressive infection is rare, though some people are at higher risk.[newsweek.com]
  • Don’t delay first aid of even minor, non-infected wounds (like blisters, scrapes, or any break in the skin).[cdc.gov]
  • Blisters, ulcers, and black spots may be present on the skin and fever, the chills, being overly tired or throwing up are also symptoms of necrotizing fasciitis.[ctdermgroup.com]
  • Over the next 24 to 48 hours the rash darkens, and blisters begin to form. Gangrene sets in, and the subcutaneous tissues become necrotic.[britannica.com]
Skin Lesion
  • Blisters, bumps, black dots, or other skin lesions might appear. In the early stages of the infection, the pain will be much worse than it looks.[healthline.com]
  • Dermatology ANATOMY & PHYSIOLOGY General High-Yield Topics Skin Anatomy and Wound Healing Terminology of Skin Lesions Wounds Raynaud Phenomenon CLINICAL CONDITIONS Bacterial Skin Infections Acne Vulgaris Acne Rosacea Cellulitis Erysipelas Impetigo Staphylococcal[medbullets.com]
  • The family did not notice other skin lesions or trauma. The patient did have a maximum temperature of 39.8 C 2 days before admission.[pediatrics.aappublications.org]
  • Necrotizing spider bites (e.g. the brown recluse spider) may produce necrotizing skin lesions that mimic gangrenous cellulitis.[infectiousdiseaseadvisor.com]
  • These infections are usually secondary, because they manifest after skin lesions due to any kind of trauma or surgical intervention. 5,28 The contributing factors are excessive pre-operative hospitalization, shaving the day before operation, elective[woundsresearch.com]
Skin Ulcer
  • Pyoderma gangrenosum is a rare noninfectious cutaneous disease characterized by expanding areas of skin ulceration around necrotic centers with purulent debris.[ncbi.nlm.nih.gov]
  • Superficial skin ulceration reached the umbilicus. After initial resuscitation, she was catheterized with a Foley catheter and intravenous ceftriaxone was given. Debridement of the wound was performed under general anaesthesia.[emro.who.int]
  • Fever and chills Fatigue Progressive or later symptoms include the following: Progressive skin changes such as skin ulceration and bullae (thin-walled fluid-filled blisters ) formation Skin discoloration Necrotic scars (black scabs) Gas formation in the[medicinenet.com]
  • Other predisposing factors include trauma, fish-fin injury, chronic skin ulcer, burns, post operative wound infection, insect bite and colo-cutaneous fistula [ 24 – 27 ].[sjtrem.biomedcentral.com]
  • Painful skin ulcers with gangrenous margins may be a feature of mixed bacterial infections. 2 The presence of crepitus is variable.[aafp.org]
Confusion
  • Necrotizing fasciitis is reported in half of patients with streptococcal toxic shock-like syndrome, or STSS. This illness is characterized by the presence of multiple symptoms: chills, fever or hypothermia and shock, confusion, vomiting, diarrhea, tachycardia[health.state.mn.us]
  • Later symptoms can include: Fever Chills Fatigue (tiredness) Vomiting These confusing symptoms may delay a person from seeking medical attention. If you have these symptoms after a wound, see a doctor right away.[cdc.gov]
  • "It's difficult to diagnose necrotizing fasciitis, because in the early stages, the signs and symptoms tend to be very wide, very general, very easily confused with minor diseases and minor conditions," said study researcher Dr.[huffingtonpost.com]
  • Flu like symptoms begin to occur, such as diarrhea, nausea, fever, confusion, dizziness, weakness, and general malaise; 5. Intense thirst occurs as the body becomes dehydrated; 6. The biggest symptom is all of these symptoms combined.[healthieryou.com]
  • This is because the symptoms — fever, pain, swelling and tenderness at the affected site — may be non-specific or confused with a mild, superficial infection.[abc.net.au]
Altered Mental Status
  • Average 3.0 of 22 Ratings Question COMMENTS (10) (OBQ04.264) A 56-year-old diabetic male presents to the emergency department by ambulance after developing high-grade fevers, malaise, and altered mental status.[orthobullets.com]
  • mental status Systemic inflammatory response syndrome Fulminant shock and multiorgan failure Body sites most often affected by necrotizing fasciitis: Lower extremities Perineum and genitourinary region (Fournier's gangrene) Upper extremities Abdomen[clinicaladvisor.com]
  • Patients are acutely ill, with high fever, tachycardia, altered mental status ranging from confusion to obtundation, and hypotension. Patients may be bacteremic or septic and may require aggressive hemodynamic support.[merckmanuals.com]
  • Patients must be operated on emergently if they present with altered mental status, hypotension, left shift or metabolic acidosis. [36] Early surgical intervention is life-saving and must be performed as early as possible, since a delay in treatment beyond[eyewiki.aao.org]

Workup

The following investigations are helpful in diagnosing necrotizing fasciitis.

  • Blood tests for white blood cell count, serum sodium levels, C-reactive protein and creatine kinase.
  • Blood urea nitrogen (BUN)
  • Arterial blood gas measurement
  • Bedside finger test: If the skin is open, the index finger can easily pass through the skin layers or if the skin is not broken, a scalpel can be used to perform this test.
  • Blood culture
  • Urinalysis
  • Wound swab culture
  • Tissue biopsy
  • Frozen tissue biopsy
  • Gram staining of the cultures

Imaging techniques for the investigation of necrotizing fasciitis include the following.

  • Soft tissue radiograph
  • Computerized tomography (CT) scan
  • Magnetic resonance imaging (MRI)
  • Ultrasound
  • Infrared spectroscopy for measuring the tissues oxygen saturation
Abnormal Renal Function
  • As in severe sepsis, abnormal renal function, hypoalbuminaemia, hyponatraemia, abnormal liver function, metabolic acidosis, and high serum lactate concentrations may occur.[academic.oup.com]
  • Blood tests - may show leukocytosis, acidosis, altered coagulation profile, hypoalbuminaemia and abnormal renal function. [ 5 ] ( NB : an inflammatory response may be absent in patients who are immunosuppressed or with liver disease.)[patient.info]
Vibrio Vulnificus
  • Fortunately, Vibrio vulnificus infection is relatively uncommon, but the incidence seems to be increasing. The U.S.[medicinenet.com]
  • Vibrio vulnificus infection often occurs in warm regions, frequently leading to necrotizing fasciitis, sepsis, and death.[ncbi.nlm.nih.gov]
  • The most recent case that made national headlines involved a man who died four days after becoming infected with the ocean-dwelling microbe Vibrio vulnificus .[acsh.org]
  • The disease is caused by certain bacteria including the following: Streptococci (group A, Streptococcus pyogenes) Methicillin resistant Staphylococcus aureus Clostridium perfringens Bacteroides fragilis Aeromonas hydrophila Vibrio vulnificus Pseudomonas[symptoma.com]
  • It was not possible to identify the specific strain, but one — Vibrio vulnificus — is known to cause necrotizing fasciitis, said Charlotte Smith, an environmental health expert at the University of California, Berkeley’s School of Public Health who helped[nytimes.com]
Gram-Positive Coccus
  • Cause of Necrotizing Fasciitis Group A Strep Streptococcus pyogenes , also known as group A beta-hemolytic Streptococcus, or group A strep (GAS) is a gram-positive coccus (spherical bacteria) that is ubiquitous, highly communicable, and spread primarily[emedicinehealth.com]

Treatment

The treatment of necrotizing fasciitis consists of the following.

  • Management of symptoms of shock (by the administration of intravenous fluids and provision of intensive care)
  • Intravenous wide-spectrum antibiotics are given. These include benzyl penicillin with clindamycin and gentamicin, or meropenem and clindamycin, or clindamycin, ciprofloxacin and metronidazole, or tetracycline and third-generation cephalosporins ( such as doxycycline, ceftazidime) or piperacillin/tazobactam.
  • Intravenous Immunoglobulins can also be given in these patients. [6]
  • Surgical debridement or in extreme cases, amputation of the affected part may also be required.
  • VAC (Vacuum Assisted Closure) is currently a popular option for the treatment of necrotizing fasciitis [7] [8].
  • Hyperbaric oxygen therapy has also shown good results in the management of necrotizing fasciitis [9] [10].
  • Skin grafts may be required for surgical or cosmetic benefit in these patients.

Prognosis

The morbidity and mortality rates associated with necrotizing fasciitis are high. If prompt treatment is not given, limb amputation will be required. A mortality rate of 20% to 75% has been found. Permanent disfigurement is the commonest complication of majority of the cases. Average life span of these patients has been found to be 38 to 44 years.

Etiology

Necrotizing fasciitis is common in cases of chronic infections (chronic liver disease, chronic renal disease) or in immunocompromised individuals, for example, in diabetics, HIV patients, those undergoing cancer chemotherapy or organ transplant patients. Bacteria enter the body through a break in the skin like cuts, abrasions, lacerations or surgical wounds.

The disease is caused by certain bacteria including the following [1] [2]:

It may also be due to opportunistic fungal infections inclding:

Based on etiology, necrotizing fasciitis is classified into following categories:

  • Type I (polymicrobial, more than one type of bacteria are involved, may be aerobic or anaerobic)
  • Type II (monomicrobial, Group A streptococcal infection, occasionally, staphylococci are involved)
  • Type III (monomicrobial, gram negative bacteria are involved).
  • Type IV (fungal infection)

Epidemiology

The incidence of the disease is 1:10,000. It is more common in Asian and African countries. A male dominance with a 2-3:1 ratio has been recorded. The disease is rare in children.

The incidence is higher in immunocompromised patients, patients with neutropenia, individuals harboring open wounds, in the elderly, pregnant women and in obese people.

Sex distribution
Age distribution

Pathophysiology

The toxins (most commonly those released by group A beta hemolytic Streptococci (GABS) and Streptococcal pyrogenic exotoxins (SPEs) A, B, and C) released by the aerobic bacteria breakdown the skin and subcutaneous tissues, invading the deeper structures [3]. They cause the inhibition of immune response of the host and also cause tissue hypoxia, causing the gram negative bacteria to grow into the infected tissues [4]. Vascular occlusion and ischemia follow. The infected tissue eventually begins to die (necrosis) [5]. The bacteria enter the blood stream eventually, causing sepsis and shock.

Prevention

The following preventive measures are effective in reducing the occurrence of necrotizing fasciitis.

  • Proper tending of open cuts or wounds.
  • Washing hands regularly with soap and plenty of water.
  • Avoiding swimming or other such activities in case of a wound or infection till it heals.
  • Aseptic techniques should be used during surgeries to prevent infection of the wounds
  • Post-op care should be provided to the patients to avoid secondary MRSA infections.

Summary

Necrotizing fasciitis refers to the acute infection and necrosis of the fascia, the layer of connective tissue that surrounds most of the body tissues, like muscles, vessels and nerves. The infection is caused by certain bacteria that seem to “eat” the skin and subcutaneous structures of the body, resulting in a grotesque appearance, hence the name “flesh eating disease”.

Patient Information

Necrotizing fasciitis, commonly known as “flesh eating disease” is the disorder in which bacteria enter the body through open wounds. Individuals, in whom the immune defense mechanisms are low, are particularly susceptible to the disease. The bacteria “eat” the skin and the underlying tissues. The structures underneath are exposed.

If infection spreads to a large area and prompt medical care is not provided to the patient, amputation of the affected organ may be required. The patient may even die if not given adequate treatment. As bacteria enter through the breaks in the skin, it is vital to keep all sorts of wounds, cuts and abrasions clean. With proper care, the disease can be prevented.

References

Article

  1. Nakamura S, Nakayama K, Mikami H, Imai T. Multiple necrotizing fasciitis: its etiology and histopathological features. The Journal of dermatology. Dec 1987;14(6):604-608.
  2. Galosi A, Luttiken R, Enderer K. [Etiology and diagnosis of necrotizing fasciitis (author's transl)]. Zeitschrift fur Hautkrankheiten. Jan 15 1981;56(2):118-125.
  3. Fink A, DeLuca G. Necrotizing fasciitis: pathophysiology and treatment. Medsurg nursing : official journal of the Academy of Medical-Surgical Nurses. Feb 2002;11(1):33-36.
  4. Young MH, Aronoff DM, Engleberg NC. Necrotizing fasciitis: pathogenesis and treatment. Expert review of anti-infective therapy. Apr 2005;3(2):279-294.
  5. McGee EJ. Necrotizing fasciitis: review of pathophysiology, diagnosis, and treatment. Critical care nursing quarterly. Jan-Mar 2005;28(1):80-84.
  6. Cawley MJ, Briggs M, Haith LR, Jr., et al. Intravenous immunoglobulin as adjunctive treatment for streptococcal toxic shock syndrome associated with necrotizing fasciitis: case report and review. Pharmacotherapy. Sep 1999;19(9):1094-1098.
  7. Novelli G, Catanzaro S, Canzi G, Sozzi D, Bozzetti A. Vacuum assisted closure therapy in the management of cervico-facial necrotizing fasciitis: a case report and review of the literature. Minerva stomatologica. Apr 2014;63(4):135-144.
  8. de Geus HR, van der Klooster JM. Vacuum-assisted closure in the treatment of large skin defects due to necrotizing fasciitis. Intensive care medicine. Apr 2005;31(4):601.
  9. Hirn M. Hyperbaric oxygen in the treatment of gas gangrene and perineal necrotizing fasciitis. A clinical and experimental study. The European journal of surgery. Supplement. : = Acta chirurgica. Supplement. 1993(570):1-36.
  10. Krasova Z, Matusek A, Chmelar D. [Hyperbaric oxygenation in the treatment of necrotizing fasciitis]. Vnitrni lekarstvi. Jul 1992;38(7):640-644.

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