notes by dr Claudio Italiano
Waterhouse-Friderichsen syndrome is a rare but dangerous pathology that comes
in the form of a fulminant septicemia, which means that the bacteria enter the
bloodstream and cause a serious infection. The syndrome was first described by
doctors Rupert Waterhouse (1873-1958), an English doctor, and Carl Friderichsen
(1886-1979), a Danish pediatrician.
It is characterized by secondary adrenal insufficiency due to a severe bacterial
infection classically associated with Neisseria meningitidis sepsis, but which
occasionally can also be caused by staphylococci, Haemophilus influenzae,
Pseudomonas
• Rapidly progressive hypotension leading to septic shock
• Intravascular coagulation disseminated with purple spread especially at the
level of the skin
• Rapid development of adrenal insufficiency, associated with massive bilateral
adrenal hemorrhage
Waterhouse-Friderichsen syndrome can be caused by many different organisms, as we said. . When it is caused by Neisseria meningitidis, WFS is considered the most severe form of meningococcal sepsis. The onset of the disease is nonspecific with fever, neck stiffness, vomiting and headache. Soon an exagger appears; first macular, not very different from pink spots of typhus, and quickly becoming petechial and purple with a dark gray color. Low blood pressure (hypotension) develops and leads rapidly to septic shock. The cyanosis of the extremities can be extreme and the patient is very prostrate or in a coma. In this form of meningococcal disease, meningitis generally does not occur. Low levels of glucose and sodium in the blood, high blood potassium levels and ACTH stimulation tests demonstrate acute adrenal insufficiency. Leukocytosis should not be extreme and in fact one can observe a leukopenia and is a very poor prognostic sign. C-reactive protein levels can be high or almost normal. Thrombocytopenia is sometimes extreme, with changes in prothrombin time (PT) and part time thromboplastin (PTT) suggesting a disseminated intravascular coagulation (DIC). Acidosis and acute renal failure can be seen as in any severe sepsis. Meningococci can be readily picked up by blood or cerebrospinal fluid and can sometimes be seen in strips of skin lesions. Also the difficulties of swallowing, atrophy of the tongue and cracks at the corners of the mouth are peculiar characteristics.
The symptomatology manifests itself with multiple bleeding to the skin, fever,
adrenal hemorrhages on both sides and collapse, while the diagnosis as well as
with the history of the symptoms, is carried out through blood tests and other
specific tests.
It can occur at any age, even if it shows a greater incidence in childhood, the
origin of adrenal hemorrhage is uncertain even if it could be attributed to the
direct growth of bacteria in the small vessels of the adrenal gland, to the
development of disseminated intravascular coagulation, endotoxin vasculitis or
some hypersensitivity vasculitis. Whatever the origin the adrenals become
coagulated blood bags, with the cancellation of the previous structure.
Histology shows that hemorrhage begins in the medulla near the thin-walled
venous sinusoids, after it extends peripherally towards the cortex, often
sparing the islands of well recognizable cortical cells.
Routine vaccination against meningococcus is recommended by centers for disease
control and prevention for all 11- 18 years of age and people with poor spleen
function (who, for example, have had their spleen removed or who have sickle
cell anemia, which damages the spleen), or that have certain immune disorders,
such as a complement deficiency.
If the disease is quickly recognized and treated effectively with antibiotics
and supporting the vital functions of the patient, healing is possible even if
the clinical picture is very compromised, otherwise the patient dies within a
few days or even a few hours, especially in age childish. Benzylpenicillin was
once the drug of choice with chloramphenicol as a good alternative in allergic
patients. Ceftriaxone is an antibiotic commonly used today. Hydrocortisone can
sometimes reverse adrenal insufficiency. Amputation, reconstructive surgery and
tissue grafting are sometimes necessary due to tissue necrosis