Synonym(s)
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Peracute bacterial sepsis with microcirculatory disorders, disseminated intravascular coagulation and shock (adrenal insufficiency due to hemorrhagic infarcts) as well as skin and adrenal hemorrhages. The incubation period is 3-4 days. Untreated, the mortality rate is 80%.
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EtiopathogenesisThis section has been translated automatically.
The most frequent triggers are infections with Neisseria meningitidis, but also septic pneumococcal or staphylococcal infections with endotoxin release and resulting excessive consumption of protein C, consecutive consumption coagulopathy and microthromboses (see below disseminated intravascular coagulopathy). Occurs particularly in splenectomized patients.
In rare cases, the syndrome has been observed after varicella infection (Heitz AFN et al. 2017)
Clinical featuresThis section has been translated automatically.
Peracute onset of illness, nausea, fever, headache, shock, symmetrical purpura(purpura fulminans), ecchymoses and sugillations, impaired consciousness up to coma and seizures. Intravital cadaveric spots.
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TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
Lethality 15-20%. Untreated, the mortality rate is 80%. Prognosis is rather unfavourable in case of extensive skin infestation.
ProphylaxisThis section has been translated automatically.
For splenectomized patients, the STIKO recommends vaccination with a conjugated meningococcal c vaccine, as well as for all children from the age of 12 months.
Note(s)This section has been translated automatically.
There is an obligation to report suspected illness, pathogen detection, illness or death.
LiteratureThis section has been translated automatically.
- Friderichsen C (1918) Adrenal apoplexy in young children. Jb Pediatrics 87: 109-125
- Heitz AFN et al (2017) A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster
infection.Neth J Med 75:351-353. - Karakousis PC et al (2001) Waterhouse-Friderichsen syndrome after infection with group A streptococcus. Mayo Clin Proc 76: 1167-1670
- Lewis LS (1979) Prognostic factors in acute meningococcaemia. Arch Dis Child 54: 44-48
- Little EGG (1901) Cases of purpura, ending fatally, associated with hemorrage into the suprarenal capsules. Brit J Derm 13: 445
- Marchand F (1880) About a peculiar disease of the sympathicus, the adrenal glands, the peripheral nerves without bronchial skin. Virchows Arch path Anat 81: 477-502
- Marinescu G (1976) On the clinical pathology of Waterhouse-Friderichsen syndrome. Münch med Wschr 118: 31-34
- Tsokos M (2003) Fatal Waterhouse-Friderichsen syndrome due to Ewingella americana infection. On J Forensic Med Pathol 24: 41-44
- Waterhouse R (1911) A case of suprarenal apoplexia. Lancet II: 577-578
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Incoming links (17)
Addison's disease; Adrenal apoplexy; Adrenal cortex apoplexy; Adrenal hemorrhage syndrome; Adrenocortical insufficiency, acute; Friderichsen-waterhouse syndrome; Fulminating purple meningococcemia; Meningococcal sepsis, fulminant; Meningococcal sepsis, fulminant or peracute; Meningococcic adrenal syndrome; ... Show allOutgoing links (15)
Antibiotics; Disseminated intravascular coagulation; Ecchymoses; Heparins systemic; Neisseria meningitidis ; Penicillin g; Pneumococci; Protein c; Purpura fulminans; Purpura (overview); ... Show allDisclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Authors
Last updated on: 26.11.2024
Author: 
Prof. Dr. med. Peter Altmeyer
Editors: 
Evgenia Bauer Dr. Christina Spiegelfeld
