A 30-Year-Old With a Full-Body Rash, Vomiting, and Confusion

Andrea Bianchin, MD; Moreno Agostini, MD


September 22, 2021

The diagnosis of Waterhouse-Friderichsen syndrome (or of meningococcal sepsis in general) must be made early and should be based on the clinical features of fever, purpuric rash, altered mental status, meningeal signs, hypotension, and septic shock. Specific testing should be used for confirmation and for guiding subsequent treatment, but it should not delay the initiation of therapy. An effort should be made to obtain blood cultures before administering antibiotics to the patient. The results are not typically available for 12-24 hours; however, they may guide subsequent therapy. Lumbar puncture should be performed early in the course of treatment, but it should not delay the administration of antibiotics. Cerebrospinal fluid analysis should include a cell count and differential, culture, Gram stain, and protein and glucose concentrations.

The presence of meningitis is identified by leukocytosis with polymorphonuclear predominance, an elevated protein concentration, and a low glucose concentration. Gram stains are often negative in meningitis. Cultures may also be obtained from synovial, pleural, or pericardial fluid, if appropriate.

Methods for identifying meningococcus other than culture are available, such as antigen detection from biological fluids. This method is rapid and can provide an exact identification of the serogroup, but it commonly produces false-negative results. Polymerase chain reaction can also identify specific serogroups and does not require the presence of a live organism.[4]

Without prompt treatment, the mortality rate of Waterhouse-Friderichsen syndrome approaches 100%. Even with rapid and optimal medical therapy, approximately 40% of patients with meningococcal sepsis do not survive. When DIC is present, the mortality rate is as high as 90%. The overall mortality of meningococcal disease is 10%-20%, with the same percentage of survivors having permanent neurologic sequelae (ie, neurologic disability, loss of a limb, and hearing impairment).[4,5]

The most important therapeutic point for meningococcal infection is early administration of appropriate antibiotics. Empirical antibiotics should be administered whenever meningococcal infection is suspected because delays in therapy expose patients to risk for severe illness, permanent disability, or death.

Common antimicrobial agents are active against Neisseria species. Penicillin G is usually the first-line antibiotic therapy; it has a low prevalence of resistance. In areas where penicillin-resistant strains have been identified, such as the United Kingdom or Spain, a third-generation cephalosporin can be used instead. Initial treatment with broad-spectrum antibiotics is recommended in all sepsis cases because antibiotic treatment can later be changed once a specific organism is identified.

Early goal-directed therapy should be initiated, with a particular focus on fluid administration and maintenance of adequate blood pressure. The efficacy of corticosteroid treatment is controversial, but it is typically recommended in cases of sepsis and meningitis. Debridement of skin and subcutaneous tissues, with subsequent skin grafting or limb amputation, may be necessary if septicemia results in peripheral hypoperfusion with skin and bone necrosis.

In cases of fulminant meningococcemia, patients should be immediately transferred to an intensive care unit for aggressive fluid therapy, vasopressor support, and intensive hemodynamic monitoring. Activated protein C may be of use in a very limited number of patients.

Treatment of DIC includes administration of fresh frozen plasma. Additional treatments currently under investigation include monoclonal antibodies to inflammatory mediators, such as endotoxins, tumor necrosis factor, interleukins, and interferon gamma.[4,5]


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