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Waterhouse friderichsen syndromeA39.1+E35.1

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.03.2021

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Synonym(s)

acute adrenocortical insufficiency; Adrenal apoplexy; Adrenal cortex apoplexy; Adrenal hemorrhage syndrome; Adrenocortical insufficiency acute; Friderichsen-Waterhouse Syndrome; Fulminant or peracute meningococcal sepsis; Fulminating purple meningococcemia; meningococcal episode fulminante; Meningococcal sepsis fulminante or peracute; Meningococcic adrenal syndrome; Waterhouse Friderichsen's disease

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HistoryThis section has been translated automatically.

Marchand, 1880; Waterhouse, 1911; Friedrichsen, 1917

DefinitionThis section has been translated automatically.

Peracute bacterial sepsis with microcirculation disorders, disseminated intravascular coagulation and shock (adrenal insufficiency due to hemorrhagic infarction) as well as skin and adrenal bleeding. The incubation period is 3-4 days. Untreated mortality is 80%.

EtiopathogenesisThis section has been translated automatically.

Neisseria meningitidis is the most frequent trigger; however, septic pneumococcal or staphylococcal infections with endotoxin release and resulting excessive consumption of protein C, consecutive consumption coagulopathy and microthrombosis (see below disseminated intravascular coagulopathy) also occur. Especially occurring 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 the disease, nausea, fever, headache, shock, symmetric purpura ( purpura fulminans), ecchymosis and suggestion, disturbance of consciousness up to coma and seizures. Intravital lividity.

DiagnosisThis section has been translated automatically.

Frequent detection of meningococci in cerebrospinal fluid without pleocytosis. Detection of protein C deficiency (20-25% of normal value).

TherapyThis section has been translated automatically.

Intensive medical care. Stage-appropriate shock treatment. Antibiotics for meningococcal sepsis, e.g. benzylpenicillin as antibiotic of choice, heparin i.v. in the early stages (platelets > 50,000/μl). Later AT III concentrates and/or fresh frozen plasma (FFP).

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 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.

  1. Friderichsen C (1918) Adrenal apoplexy in young children. Jb Pediatrics 87: 109-125
  2. Heitz AFN et al (2017) A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster
    infection.Neth J Med 75:351-353.
  3. Karakousis PC et al (2001) Waterhouse-Friderichsen syndrome after infection with group A streptococcus. Mayo Clin Proc 76: 1167-1670
  4. Lewis LS (1979) Prognostic factors in acute meningococcaemia. Arch Dis Child 54: 44-48
  5. Little EGG (1901) Cases of purpura, ending fatally, associated with hemorrage into the suprarenal capsules. Brit J Derm 13: 445
  6. 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
  7. Marinescu G (1976) On the clinical pathology of Waterhouse-Friderichsen syndrome. Münch med Wschr 118: 31-34
  8. Tsokos M (2003) Fatal Waterhouse-Friderichsen syndrome due to Ewingella americana infection. On J Forensic Med Pathol 24: 41-44
  9. Waterhouse R (1911) A case of suprarenal apoplexia. Lancet II: 577-578

Authors

Last updated on: 21.03.2021

Author: Prof. Dr. med. Peter Altmeyer

Editors: Evgenia BauerDr. Christina Spiegelfeld