Waterhouse–Friderichsen syndrome can be caused by a number of different organisms (see below). When caused by
Neisseria meningitidis, WFS is considered the most severe form of meningococcal
sepsis. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Soon a rash appears; first macular, not much different from the rose spots of
typhoid, and rapidly becoming
petechial and
purpuric with a dusky gray color. Low blood pressure (
hypotension) develops and rapidly leads to
septic shock. The
cyanosis of extremities can be extreme and the patient is very prostrated or comatose. In this form of meningococcal disease,
meningitis generally does not occur. Low levels of
blood glucose and
sodium,
high levels of potassium in the blood, and the
ACTH stimulation test demonstrate the acute adrenal failure.
Leukocytosis need not be extreme and in fact
leukopenia may be seen and it is a very poor prognostic sign.
C-reactive protein levels can be elevated or almost normal.
Thrombocytopenia is sometimes extreme, with alteration in
prothrombin time (PT) and
partial thromboplastin time (PTT) suggestive of
disseminated intravascular coagulation (DIC). Acidosis and acute kidney failure can be seen as in any severe sepsis. Meningococci can be readily cultured from blood or
cerebrospinal fluid, and can sometimes be seen in smears of cutaneous lesions. Difficulty swallowing, atrophy of the tongue, and cracks at the corners of the mouth are also characteristic features. ==Causes==