Dermatologic Manifestations of Meningococcemia 

  • Author: Elizabeth L Tanzi, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 16, 2011
 

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.

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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).

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Epidemiology

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.

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Contributor Information and Disclosures
Author

Elizabeth L Tanzi, MD  Co-Director, Laser Surgery, Washington Institute of Dermatologic Laser Surgery; Assistant Professor, Department of Dermatology, Johns Hopkins University School of Medicine

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 College of Physicians and Surgeons; Director of Pediatric Dermatology, Department of Dermatology, St Luke's Roosevelt Hospital Center, Maimonides Medical Center and Beth Israel Medical Center

Nanette Silverberg 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.

Specialty Editor Board

Gregory J Raugi, MD, PhD  Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; 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.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

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.

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 Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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