Waterhouse friderichsen syndrome A39.1+E35.1

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 21.03.2021

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

History
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Marchand, 1880; Waterhouse, 1911; Friedrichsen, 1917

Definition
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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%.

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Purpura fulminans

Rare acquired syndrome following an initial infectious episode with consumption coagulopathy, haemorrhages ...

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Etiopathogenesis
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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 features
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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.

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

Therapy
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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/forecast
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Lethality 15-20%. Untreated, the mortality rate is 80%. Prognosis is rather unfavourable in case of extensive skin infestation.

Prophylaxis
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For splenectomized patients, the STIKO recommends vaccination with a conjugated meningococcal c vaccine for all children from the age of 12 months.

Note(s)
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There is an obligation to report suspected illness, pathogen detection, illness or death.

Literature
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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