eMedicine Specialties > Dermatology > Bacterial Infections

Meningococcemia

Author: Elizabeth L Tanzi, MD, Co-Director, Laser Surgery, Washington Institute of Dermatologic Laser Surgery
Coauthor(s): Nanette Silverberg, MD, Assistant Clinical Professor, Department of Dermatology, Columbia University School of Medicine; Director of Pediatric Dermatology, Department of Dermatology, St Luke's Roosevelt Hospital Center, Maimonides Medical Center and Beth Israel Medical Center
Contributor Information and Disclosures

Updated: Sep 11, 2009

Introduction

Background

Meningococcal disease is a communicable infection caused by Neisseria meningitidis. Meningococcemia is transmitted from person to person via respiratory secretions. N meningitidis infection can be clinically polymorphic. The most common disease presentation is meningitis, but this is not always the case.1 Rarely, N meningitidis infection may manifest as chronic meningococcemia that resembles the arthritis-dermatitis syndrome of gonococcemia; however, acute meningococcal septicemia (also called meningococcemia) is the most devastating form of the disease.

Meningococcemia can kill more rapidly than any other infectious disease. Early recognition is critical to implement prompt antibiotic therapy and supportive care.2 Treatment must be instituted rapidly because irreversible shock and death may occur within hours of the onset of symptoms. Cutaneous manifestations in meningococcemia may be important clues to the diagnosis. Skin involvement can be the most dramatic aspect of the disease and is often the first sign that leads to the clinical consideration of meningococcemia.

Pathophysiology

N meningitidis is an obligate, nonmotile, aerobic, encapsulated, gram-negative diplococcus that can only be cultured on blood-enriched media in a 5-10% carbon dioxide–enriched environment. The outer polysaccharide capsule of N meningitidis serves as the basis of serologic grouping. To date, at least 13 different serogroups have been identified; however, groups A, B, C, Y, and W-135 are the major pathogens involved in human disease.3,4

Transmission of N meningitidis occurs from person to person through respiratory secretions. The human upper respiratory tract is the only known reservoir. Carrier rates depend on age. Approximately 2% of children younger than 2 years, 5% of children up to 17 years, and 20-40% of young adults are carriers of N meningitidis. Overcrowded conditions (eg, schools, military camps) can significantly increase the carrier rate. Screening of military recruits performed during recent epidemics demonstrated that although as many as 95% of recruits were oropharyngeal carriers, only 1% developed systemic disease. Because very few recruits with meningococcal disease had ever been in contact with another such patient, asymptomatic carriers are thought to be the major source of transmission of pathogenic strains.

A complex interaction between host factors and the organism determines the outcome of exposure to N meningitidis. Colonization and invasion of meningococci are facilitated by pili that attach to mucosal epithelial cells. A concomitant viral infection may facilitate the invasion of N meningitidis into the bloodstream or lower respiratory tract. Once in the bloodstream, N meningitidis causes profound effects on small blood vessels, related to both direct invasion of endothelial cells and indirect damage from endotoxin release. Endotoxin from the lipopolysaccharide of meningococci causes endothelial cells, monocytes, and macrophages to release tumor necrosis factor-alpha, interleukin 1, interleukin 6, and interferon-gamma.5

The deleterious effects of these cytokines play a major role in the pathogenesis of meningococcemia by causing severe hypotension, reduced cardiac output, and increased endothelial permeability. Multiple organ failure, shock, and death may ensue as a result of anoxia in vital organs and massive disseminated intravascular coagulation (DIC).

Frequency

United States

An estimated 2600 cases of meningococcemia occur each year.

Mortality/Morbidity

The mortality rate for meningococcemia is approximately 5% in children and 5-10% in adults; however, meningococcemia associated with DIC has a mortality rate of higher than 90%.

Age

Children younger than 4 years have the highest risk of developing meningococcal disease. Neonates are often resistant to disease because passively acquired maternal immunoglobulin G antibodies are present until approximately age 6 months. As the child grows older, asymptomatic exposure to a variety of encapsulated and nonencapsulated N meningitidis strains increases protective bacterial immunity. Protective immunoglobulin M and immunoglobulin G are found in up to 95% of young adults.

Clinical

History

  • Meningococcemia follows an upper respiratory tract infection and is associated with headache, nausea, vomiting, myalgias, and arthralgias.
  • Not all patients appear toxic.
  • The initial presentation may be difficult to distinguish from a viral syndrome.
  • While a slower clinical presentation can occur in persons with a milder form of disease, fever may increase dramatically with rapid clinical deterioration.
  • In fulminant meningococcemia, a hemorrhagic eruption, hypotension, and cardiac depression may be apparent within hours of the initial presentation.

Physical

  • Cutaneous manifestations of meningococcemia are common and can be the presenting sign of disease.
  • Petechiae are the most common sign, occurring in 50-60% of patients with meningococcemia; however, urticarial and maculopapular lesions also may occur initially. Petechiae are most often located on the extremities and trunk but may progress to involve any part of the body. Petechiae may appear in groups under areas of pressure.
  • With progression of meningococcemia, pustules, bullae, and hemorrhagic lesions with central necrosis can develop. Stellate purpura with a central gunmetal-gray hue is characteristic and should be considered highly suggestive of meningococcemia.
  • Large maplike purpuric and necrotic areas related to the development of DIC are characteristic of fulminant meningococcemia.
  • Noncutaneous physical findings are as follows:
    • Altered mental status
    • Neck stiffness
    • Irritability
    • Seizures
    • Nerve palsies
    • Gait disturbance
    • Nausea
    • Vomiting
    • Unstable vital signs

Causes

  • Most patients with meningococcal disease are previously healthy individuals; however, patients with certain medical conditions are at increased risk for developing meningococcal infection.
  • Meningococcemia is particularly common among individuals with deficiencies of terminal complement components C5-C9 or properdin.
    • These late complement components are required for bacteriolysis of meningococci.
    • An estimated 50-60% of individuals with late complement component deficiencies develop at least one episode of meningococcal disease.
    • Many of these patients experience multiple episodes of infection.
  • Acquired complement deficiencies that occur in association with systemic lupus erythematosus, multiple myeloma, severe liver disease, enteropathies, and the nephrotic syndrome also predispose to meningococcal infection.
  • An association has been described between increased risk of mortality in children with meningococcal disease and polymorphisms in the interleukin 1 cluster.
  • Other risk factors include immunoglobulin deficiency, asplenia, and HIV infection.

More on Meningococcemia

Overview: Meningococcemia
Differential Diagnoses & Workup: Meningococcemia
Treatment & Medication: Meningococcemia
Follow-up: Meningococcemia
References
[ CLOSE WINDOW ]

References

  1. Horino T, Kato T, Sato F, et al. Meningococcemia without meningitis in Japan. Intern Med. 2008;47(17):1543-7. [Medline].

  2. Razminia M, Salem Y, Elbzour M, Teves D, Deshmukh H, Khosla S. Importance of early diagnosis and therapy of acute meningococcal myocarditis: a case report with review of literature. Am J Ther. May-Jun 2005;12(3):269-71. [Medline].

  3. Jarva H, Ram S, Vogel U, Blom AM, Meri S. Binding of the complement inhibitor C4bp to serogroup B Neisseria meningitidis. J Immunol. May 15 2005;174(10):6299-307. [Medline].

  4. Nkosi J, Thakrar A, Kumar K, et al. Meningococcal serotype Y myopericarditis. Diagn Microbiol Infect Dis. Feb 2009;63(2):223-7. [Medline].

  5. Endler G, Marculescu R, Starkl P, et al. Polymorphisms in the interleukin-1 gene cluster in children and young adults with systemic meningococcemia. Clin Chem. Mar 2006;52(3):511-4. [Medline].

  6. Tuncer AM, Gur I, Ertem U, et al. Once daily ceftriaxone for meningococcemia and meningococcal meningitis. Pediatr Infect Dis J. Oct 1988;7(10):711-3. [Medline].

  7. Aiuto LT, Barone SR, Cohen PS, Boxer RA. Recombinant tissue plasminogen activator restores perfusion in meningococcal purpura fulminans. Crit Care Med. Jun 1997;25(6):1079-82. [Medline].

  8. Rivard GE, David M, Farrell C, Schwarz HP. Treatment of purpura fulminans in meningococcemia with protein C concentrate. J Pediatr. Apr 1995;126(4):646-52. [Medline].

  9. [Guideline] American Academy of Pediatrics Committee on Infectious Diseases. Prevention and control of meningococcal disease: recommendations for use of meningococcal vaccines in pediatric patients. Pediatrics. Aug 2005;116(2):496-505. [Medline].

  10. [Guideline] Centers for Disease Control and Prevention (CDC) Advisory Committee on Immunization Practices. Revised recommendations of the Advisory Committee on Immunization Practices to Vaccinate all Persons Aged 11-18 Years with Meningococcal Conjugate Vaccine. MMWR Morb Mortal Wkly Rep. Aug 10 2007;56(31):794-5. [Medline].

  11. [Guideline] Scottish Intercollegiate Guidelines Network (SIGN). Management of invasive meningococcal disease in children and young people. A national clinical guideline. National Guidelines Clearinghouse. May 2008.

  12. Herrera R, Hobar PC, Ginsburg CM. Surgical intervention for the complications of meningococcal-induced purpura fulminans. Pediatr Infect Dis J. Aug 1994;13(8):734-7. [Medline].

  13. Besner GE, Klamar JE. Integra Artificial Skin as a useful adjunct in the treatment of purpura fulminans. J Burn Care Rehabil. Jul-Aug 1998;19(4):324-9. [Medline].

  14. Faibis S, Widmer R, Sapir S, Peretz B, Shapira J. Meningococcal septicaemia and dental complications: a literature review and two case reports. Int J Paediatr Dent. May 2005;15(3):213-9. [Medline].

  15. Aaberge IS, Oster P, Helland OS, et al. Combined administration of meningococcal serogroup B outer membrane vesicle vaccine and conjugated serogroup C vaccine indicated for prevention of meningococcal disease is safe and immunogenic. Clin Diagn Lab Immunol. May 2005;12(5):599-605. [Medline].

  16. Zeidan A, Tariq S, Faltas B, Urban M, McGrody K. A case of primary meningococcal pericarditis caused by Neisseria meningitidis serotype Y with rapid evolution into cardiac tamponade. J Gen Intern Med. Sep 2008;23(9):1532-5. [Medline].

  17. Parmentier L, Garzoni C, Antille C, Kaiser L, Ninet B, Borradori L. Value of a novel Neisseria meningitidis--specific polymerase chain reaction assay in skin biopsy specimens as a diagnostic tool in chronic meningococcemia. Arch Dermatol. Jun 2008;144(6):770-3. [Medline].

  18. Centers for Disease Control and Prevention. Prevention and control of meningococcal disease. Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Recomm Rep. Jun 30 2000;49:1-10. [Medline].

  19. Darmstadt GL. Acute infectious purpura fulminans: pathogenesis and medical management. Pediatr Dermatol. May-Jun 1998;15(3):169-83. [Medline].

  20. Figueroa JE, Densen P. Infectious diseases associated with complement deficiencies. Clin Microbiol Rev. Jul 1991;4(3):359-95. [Medline].

  21. Hazelzet JA. Diagnosing meningococcemia as a cause of sepsis. Pediatr Crit Care Med. May 2005;6(3 Suppl):S50-4. [Medline].

  22. Herf C, Nichols J, Fruh S, Holloway B, Anderson CU. Meningococcal disease: recognition, treatment, and prevention. Nurse Pract. Aug 1998;23(8):30, 33-6, 39-40 passim. [Medline].

  23. Ikeda C, Capozzi A. Management of skin loss in meningococcal infection. Ann Plast Surg. Oct 1987;19(4):375-7. [Medline].

  24. Lynn WA, Cohen J. Adjunctive therapy for septic shock: a review of experimental approaches. Clin Infect Dis. Jan 1995;20(1):143-58. [Medline].

  25. Periappuram M, Taylor MR, Keane CT. Rapid detection of meningococci from petechiae in acute meningococcal infection. J Infect. Nov 1995;31(3):201-3. [Medline].

  26. Ploysangam T, Sheth AP. Chronic meningococcemia in childhood: case report and review of the literature. Pediatr Dermatol. Nov-Dec 1996;13(6):483-7. [Medline].

  27. Salzman MB, Rubin LG. Meningococcemia. Infect Dis Clin North Am. Dec 1996;10(4):709-25. [Medline].

  28. Schaller RT Jr, Schaller JF. Surgical management of life-threatening and disfiguring sequelae of fulminant meningococcemia. Am J Surg. May 1986;151(5):553-6. [Medline].

  29. van Deuren M, van Dijke BJ, Koopman RJ, et al. Rapid diagnosis of acute meningococcal infections by needle aspiration or biopsy of skin lesions. BMJ. May 8 1993;306(6887):1229-32. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

meningococcemia, meningococcal sepsis, meningococcal disease, Neisseria meningitidis, N meningitidis, meningococcal infection, meningitis, bacteremia, meningococcemia, acute meningococcal septicemia, adrenal hemorrhage, Waterhouse-Friderichsen syndrome, meningococcal septicemia

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Elizabeth L Tanzi, MD, Co-Director, Laser Surgery, Washington Institute of Dermatologic Laser Surgery
Elizabeth L Tanzi, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Nanette Silverberg, MD, Assistant Clinical Professor, Department of Dermatology, Columbia University School of Medicine; Director of Pediatric Dermatology, Department of Dermatology, St Luke's Roosevelt Hospital Center, Maimonides Medical Center and Beth Israel Medical Center
Nanette Silverberg, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, American Association of University Women, American Medical Association, American Medical Women's Association, Dermatology Foundation, International Society of Pediatric Dermatology, Phi Beta Kappa, Sigma Xi, Society for Pediatric Dermatology, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Medical Editor

Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle
Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.