September 2006
Fig.3
Fig.4
Diagnosis: Meningococcemia
Histopathology: Vasculopathic dermatitis (Fig. 3) with
intravascular gram negative diplococci (Fig. 4). Consistent the
clinical history of meningococcemia.
Tissue biopsy culture: Negative for any growth
Blood culture: 2/2 positive for Neisseria Meningitidis
Lumbar puncture: negative for organisms
Patient course:
The patient was started on ceftriaxone IV in the emergency
room and then transferred to intensive care unit. Three days later,
the patient was discharged home.
Discussion:
Meningococcal disease is a communicable infection caused by
the gram negative diplococcus, Neisseria meningitidis.
It is transmitted from person to person via respiratory secretions.
N meningitidis infection can be clinically polymorphic.
The most common disease presentation is meningitis, which is the
most devastating. Meningococcemia can kill more rapidly than any
other infectious disease. Early recognition is critical to implement
prompt antibiotic therapy and supportive care. 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, in some cases, can be the
most dramatic aspect of the disease and is often the first sign
that leads to the clinical consideration of meningococcemia. The
mortality rate is approximately 5% in children and 5-10% in adults;
however, meningococcemia associated with DIC has a mortality rate
of higher than 90%.
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. Other risk factors
include immunoglobulin deficiency, asplenia, and HIV infection.
The most important measure in treating meningococcemia is early
detection and rapid administration of antibiotics. Penicillin
G is the antibiotic of choice for susceptible isolates. A third-generation
cephalosporin (eg, cefotaxime, ceftriaxone) can be used initially
in septic patients while the diagnosis is being confirmed or in
countries where penicillin-resistant strains of N meningitidis
have been isolated.
References:
1. Herf C, Nichols J, Fruh S, et al: Meningococcal disease:
recognition, treatment, and prevention. Nurse Pract 1998 Aug;
23(8): 30, 33-6, 39-40.
2. Salzman MB, Rubin LG: Meningococcemia. Infect Dis Clin North
Am 1996 Dec; 10(4): 709-25.
3. Manchanda V, Gupta S, Bhalla P. Meningococcal disease: history,
epidemiology, pathogenesis, clinical manifestations, diagnosis,
antimicrobial susceptibility and prevention. Indian J Med Microbiol.
2006 Jan;24(1):7-19.
This case was presented by Drs. Daniel Popkin, Anne Lind, Kim
Crone and Maria Canizares.