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

Andrea Bianchin, MD; Moreno Agostini, MD

Disclosures

September 22, 2021

Discussion

Waterhouse-Friderichsen syndrome is a severe complication of Neisseria meningitidis infection. N meningitidis is a gram-negative aerobic diplococcus with a particular affinity for the bloodstream and meninges. Infection with N meningitidis may cause clinical conditions ranging from fever and bacteremia to life-threatening septic shock.

N meningitidis is a commensal bacterium in many humans that resides in the nasopharynx and colonizes up to 25% of healthy people, without causing illness. It is transmitted by aerosolized respiratory particles and secretions.

The reason why some people who carry invasive strains of the bacteria become ill whereas others do not is unclear. A particular virulence factor may be a cause but is not fully understood. Crowded living conditions, such as college dormitories and military barracks, increase the risk for transmission. Other risk factors include smoking and upper respiratory tract infection, as well as systemic diseases, such as multiple myeloma, nephrotic syndrome, or systemic lupus erythematosus.

Immune defects, deficiency of humoral immunity or the complement-mediated immune system, and anatomical or functional asplenia predispose patients to N meningitidis infection, with a relative risk of 500 in asplenic patients. Outbreaks of N meningitidis infection appear to be seasonal, occurring most often in spring and winter. The yearly incidence of the disease is about 1 case per 100,000 people.[1,2,3,4,5]

N meningitidis spreads through the inhalation of respiratory secretions transmitted by a carrier. The onset of symptoms occurs approximately 3-4 days after inoculation. The patient typically begins to have symptoms of a respiratory illness, followed by fever, headache, and vomiting; this rapidly progresses to the development of mental status changes (such as lethargy or confusion).

Meningeal infection probably results from hematogenous spread; N meningitidis can be isolated via blood cultures in approximately 75% of patients. Meningococcal sepsis with multisystem involvement, however, occurs in only one fifth of cases of meningitis caused by N meningitidis.

The mortality rate of meningococcal sepsis is very high; it is even more lethal than isolated meningococcal meningitis. Waterhouse-Friderichsen syndrome is one of the most severe complications of meningococcal infection. Although often classified by the presence of multiorgan dysfunction in the presence of meningococcal infection, the syndrome is typically more specifically defined by the presence of hemorrhage into the adrenal glands.[2,3,4]

A typical purpuric rash may be seen as a result of septicemia. This usually first appears on the trunk and lower extremities and subsequently spreads throughout the body surface. The development of purpura fulminans (PF) - a life-threatening hemorrhagic condition characterized by hypotension, disseminated intravascular coagulation (DIC), and purpura - indicates a poor prognosis.

Although the presence of PF is classic for meningococcal infection, multiple causes may be responsible and should also be considered. Three general categories of PF are recognized: acute infectious PF, idiopathic PF, and abnormalities of the coagulation system. In fact, most PF cases are caused by coagulation abnormalities. Occasionally, meningococcal infection causes only focal disease, such as conjunctivitis, septic arthritis, urethritis, purulent pericarditis, or respiratory tract infection (eg, pneumonia, epiglottitis, and otitis media).[2]

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