Specialty endocrinology ICD-9-CM 036.3 MedlinePlus 000609 | ICD-10 A39.1, E35.1 DiseasesDB 29316 eMedicine med/3009 | |
Waterhouse–Friderichsen syndrome (WFS), hemorrhagic adrenalitis or fulminant meningococcemia is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection: Typically the pathogen is the meningococcus Neisseria meningitidis.
Contents
The bacterial infection leads to massive hemorrhage into one or (usually) both adrenal glands. It is characterized by overwhelming bacterial infection meningococcemia leading to massive blood invasion, organ failure, coma, low blood pressure and shock, disseminated intravascular coagulation (DIC) with widespread purpura, rapidly developing adrenocortical insufficiency and death.
Signs and symptoms
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 septicemia. 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. There is hypoglycemia with hyponatremia and hyperkalemia, and the ACTH stimulation test demonstrates the acute adrenal failure. Leukocytosis need not to 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 renal failure can be seen as in any severe sepsis. Meningococci can be readily cultured from blood or CSF, and can sometimes be seen in smears of cutaneous lesions. Dysphagia, atrophy of the tongue, and cracks at the corners of the mouth are also characteristic features.
Causes
Multiple species of bacteria can be associated with the condition:
Viruses may also be implicated in adrenal problems:
Prevention
Routine vaccination against meningococcus is recommended by the Centers for Disease Control and Prevention for all 11- to 18-year-olds and people who have poor splenic function (who, for example, have had their spleen removed or who have sickle-cell disease which damages the spleen), or who have certain immune disorders, such as a complement deficiency.
Treatment
Fulminant meningococcemia is a medical emergency and needs to be treated with adequate antibiotics as fast as possible. Benzylpenicillin was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Ceftriaxone is an antibiotic commonly employed today. Hydrocortisone can sometimes reverse the hypoadrenal shock. Sometimes plastic surgery and grafting is needed to deal with tissue necrosis.
Historical
Waterhouse–Friderichsen syndrome is named after Rupert Waterhouse (1873–1958), an English physician, and Carl Friderichsen (1886–1979), a Danish pediatrician, who wrote papers on the syndrome, which had been previously described.