Features of the WFS:
(1) septic shock
(2) cutaneous manifestations with purpura, petechiae and/or hemorrhagic bullae
(3) bilateral hemorrhage into the adrenal glands which can result in acute adrenal insufficiency
The mechanism of the fulminant course has been ascribed to:
(1) disseminated intravascular coagulation (DIC)
(2) endotoxinemia
While classically associated with meningococcemia, WFS can occur with many bacteria including Streptococcus pneumoniae, Hemophilus influenzae, E. coli and other bacteria.
Patients with hyposplenism may be at increased risk.
Management can be complex due to the fulminant nature of the disorder. Intravenous dexamethasone is usually given is the diagnosis is suspected.
The diagnosis may be missed if:
(1) The association with other bacteria other than Neisseria meningitidis is not recognized.
(2) Cutaneous manifestations are lacking.
(3) The adrenal glands are not studied on imaging studies.
(4) The clinician is unfamiliar with the syndrome.
