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Genetic and Rare Diseases Information Center (GARD)

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Waterhouse–Friderichsen syndrome


Other Names for this Disease
  • Fatal pneumococcal Waterhouse-Friderichsen syndrome
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What is Waterhouse–Friderichsen syndrome?

Waterhouse–Friderichsen syndrome is adrenal gland failure due to bleeding into the adrenal gland. It is caused by severe meningococcal infection or other severe, bacterial infection. Symptoms include acute adrenal gland insufficiency, and profound shock.[1] Most patients with this condition are children, although adults may rarely be affected.[2] It is deadly if not treated immediately.[1]

Last updated: 1/8/2010

What are the symptoms of Waterhouse-Friderichsen syndrome?

Waterhouse-Friderichsen syndrome is characterized by the abrupt onset of fever, petechiaeseptic shock, and disseminated intravascular coagulation (DIC) followed by acute hemorrhagic necrosis of the adrenal glands and severe cardiovascular dysfunction.[3][4] Patients often experience prodromic, nonspecific symptoms, including malaise, headache, weakness, dizziness, cough, arthralgia (joint pain), and myalgia (muscle pain).[2][3] 

A characteristic skin rash with a typical evolution occurs in approximately 75% of patients with Waterhouse-Friderichsen syndrome.[2]

  • In its early stages, the rash consists of small, pink macules or papules.
  • These are rapidly followed by petechial lesions, which gradually transform into large, purpuric, coalescent plaques in late stages of the disease.
Last updated: 1/8/2010

What causes Waterhouse-Friderichsen syndrome?

Waterhouse-Friderichsen syndrome is most often associated with meningococcal disease (accounts for 80% of cases). The syndrome also has been associated with other bacterial pathogens, including Streptococcus pneumoniae, group A beta-hemolytic streptococci, Neisseria gonorrhoeae, Escherichia coli, Klebsiella pneumoniae, Haemophilus influenzae (group B), Salmonella choleraesuis, Pasteurella multocida, Acinetobacter calcoaceticus, and Plesiomonas shigelloides.[2][3] It may also be associated with a history of splenectomy.[3]While the exact mechanism of disease is not clear, activation of several cytokine mediators appears to lead to sepsis and shock.[2]

 

Last updated: 1/8/2010

How might Waterhouse-Friderichsen syndrome be treated?

Treatment may include antibiotics and glucocorticoids.  Other treatment is symptomatic and supportive.[2]
Last updated: 1/8/2010

What is the prognosis for individuals with Waterhouse-Friderichsen syndrome?

In Waterhouse-Friderichsen syndrome, the case fatality rate is 55-60%, particularly when the diagnosis is delayed. Death typically results from sepsis, despite aggressive treatment.[2]
Last updated: 1/8/2010

References