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Waterhouse-Friderichsen Syndrome
Waterhouse Friderichsen Syndrome

Waterhouse-Friderichsen syndrome, also known as hemorrhagic adrenalitis, is a condition that describes acute adrenal failure, caused by hemorrhage within the adrenal glands after a severe bacterial infection, in particular meningococcal disease. The condition is most frequently seen in children younger than 10 years old.

Presentation

The onset of meningococcal Waterhouse-Friderichsen syndrome is characterized by non-specific signs and symptoms such as fatigue, tremor, fever, sweating, diarrhea, vomiting, headache, muscle pain and abdominal pain [9]. These symptoms ultimately progress into massive organ failure, coma, shock, low blood pressure and sepsis [10].

A characteristic macular rash develops early in the disease process and resembles the spots seen in typhoid fever. The rash later becomes petechial and purpuric and has a dusky gray color. Other distinguishing features include atrophy of the tongue, dysphagia and cracks around the corners of the mouth of the patient. Unlike other forms of meningococcal disease, meningitis is not a feature of WFS. 

As the patient deteriorates, heart rate increases and the development of low blood pressure signals the progression into septic shock. Peripheral cyanosis becomes prominent as the individual becomes confused and might fall into coma [11]. Metabolic abnormalities include hypoglycemia, hyperkalemia, hyponatremia and acidosis, alongside acute renal failure in cases of severe sepsis. Hematologic disturbances are characterized either by leukocytosis or leukopenia (a very negative prognostic factor), severe thrombocytopenia, as well as changes in prothrombin time and partial thromboplastin time, suggesting diffuse intravascular coagulation or DIC. C-reactive protein levels can be normal or elevated. Blood or CSF cultures may be positive for meningococci which can also be isolated from smears of cutaneous lesions. 

Entire Body System

  • Fever

    A38.0 Scarlet fever with otitis media A38.1 Scarlet fever with myocarditis A38.8 Scarlet fever with other complications A38.9 Scarlet fever, uncomplicated A39 Meningococcal infection A39.0 Meningococcal meningitis A39.1 Waterhouse-Friderichsen syndrome [icd10data.com]

    The case was started with the patient presenting with altered mental status and fever, and as the case unfolded, mental status and shock worsened allowing the learners to intubate and resuscitate. [jetem.org]

    The patient presented with neck pain and fevers of 2 days' duration. Computed tomography of the neck revealed a mass in the retropharyngeal space, suggesting an abscess. [ncbi.nlm.nih.gov]

  • Sepsis

    Blood cultures later confirmed Neisseria meningitidis sepsis, and a biopsy confirmed renal cortical infarction. [ncbi.nlm.nih.gov]

    […] with mv >96 hours or peripheral extracorporeal membrane oxygenation (ecmo) 871 Septicemia or severe sepsis without mv >96 hours with mcc 872 Septicemia or severe sepsis without mv >96 hours without mcc Convert A39.1 to ICD-9-CM Code History 2016 (effective [icd10data.com]

  • Falling

    COMPLICATION  Shock, extensive haemorrhage within the skin and fall into coma.  Death usually after a few hours, adrenal insufficiency being the immediate cause.  Patients who recover may suffer from extensive sloughing of the skin and loss of digits [slideshare.net]

    COMPLICATION Shock, extensive haemorrhage within the skin and fall into coma. Death usually after a few hours, adrenal insufficiency being the immediate cause. Patients who recover may suffer from extensive sloughing of the skin and loss of digits [fr.slideshare.net]

    The weakness resulted in him falling onto his back and hitting his head without losing consciousness. The patient denied numbness, bowel or bladder incontinence, or other neurological symptoms. [amjcaserep.com]

    The patient's condition deteriorated; he developed purpura along with a fall in platelet count, and died due to shock. [ncbi.nlm.nih.gov]

  • Pallor

    In 1918, Friderichsen 2 reviewed the literature and described a similar case with a fulminating course, characterized clinically by stupor, cyanosis, pallor, vomiting and a rapidly spreading purpuric eruption, and at autopsy by massive bilateral adrenal [nejm.org]

    The patient exhibits a cyanotic pallor, a pechial or purpuric rash, and sometimes large purpuric cutaneous haemorrhages often followed by necrosis and sloughing. [whonamedit.com]

    The clinical presentation includes severe cyanotic pallor and peripheral coldness due to profound vasoconstriction (often considered premortal), and generalized petechial or purpuric rash. Fever is initially moderate but subsequently becomes high. [accesspediatrics.mhmedical.com]

    Abrupt and rapid deterioration of clinical symptoms; hematological (high polymorphonuclear leukocyte counts, thrombocytopenia, evidence of DIC); biochemical (raised serum urea and creatinine levels, electrolyte disturbances), and clinical (cyanotic pallor [accessanesthesiology.mhmedical.com]

  • Fulminant Sepsis

    Mechanisms by which Pneumococcus can elicit fulminant sepsis are discussed, although there is no explanation as to why this may occur in the setting of normal splenic function. [ncbi.nlm.nih.gov]

    The diagnosis of WFS was based on the following clinical as well as morphological criteria: (a) fulminant sepsis, (b) patchy purpura of the skin as a result of disseminated intravascular coagulation (DIC), and (c) bilateral hemorrhagic necroses of the [link.springer.com]

    The earliest reports of this syndrome were from England in the 1900s, when Little made an association between fulminant sepsis and acute bilateral adrenal hemorrhage. [mayoclinicproceedings.org]

Respiratoric

  • Dyspnea

    […] eculizumab treatment: case report and review of management. 61 Friedl C...Eller P 28213751 2017 11 Streptococcus Pneumoniae Detection Long Time After Death in a Fatal Case of Waterhouse-Friderichsen Syndrome. 61 Verzeletti A...De Ferrari F 28009598 2017 12 [Dyspnea [malacards.org]

    low-grade fever, malaise, myalgia, and upper respiratory symptoms Pharyngitis, herpangina, and/or rash Neonatal meningitis Often nonspecific and without the classic triad of meningitis Early-onset : lethargy, vomiting, irritability, poor appetite, dyspnea [amboss.com]

  • Respiratory Insufficiency

    Circulatory and respiratory insufficiency necessitated intubation and urgent transfer to a pediatric intensive care center, where the infant was placed in isolation. [aerzteblatt.de]

Gastrointestinal

  • Vomiting

    Waterhouse-Friderichsen syndrome - a condition characterized by vomiting, diarrhea, extensive purpura, cyanosis, tonic-clonic convulsions, and circulatory collapse. [medical-dictionary.thefreedictionary.com]

    The patient presented with a six hour history of sudden onset epigastric pain, vomiting, chills, and rigors. A presumptive diagnosis of cholangitis with septicaemic shock was made. [ncbi.nlm.nih.gov]

  • Nausea

    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. [altmeyers.org]

    The principal manifestation of this disorder is overt shock, although other signs and symptoms include petechial rash, coagulopathy, generalized weakness, fatigue, nausea/vomiting, and confusion [2]. [amjcaserep.com]

    Children and adults Classic triad of meningitis: fever, headache, and neck stiffness ( nuchal rigidity ) Altered mental status Photophobia Nausea, vomiting Malaise Seizures If due to N. meningitidis : myalgia and possibly petechial or purpuric rash (especially [amboss.com]

    Nausea and vomiting. DDx: [17] Autoimmune. Tuberculosis. AIDS. Malignancy. Notes: Secondary adrenocortical insufficiency (due to pituitary pathology): [18] No hyperpigmentation (as no POMC). Aldosterone usu. normal. [librepathology.org]

    Patients often have nonspecific symptoms like rapid onset headache, fever, weakness, fatigue, abdominal or flank pain, anorexia, nausea or vomiting, confusion, or disorientation. [statpearls.com]

  • Abdominal Pain

    An otherwise healthy 36-year-old man had abdominal pain, vomiting, sepsis, and disseminated intravascular coagulation (DIC). Negative exploratory laparotomy was shortly followed by death. [ncbi.nlm.nih.gov]

    The pain was accompanied by the feeling of general weakness. His temperature was normal at 36.7 °C. His blood pressure was 170/90 mmHg with a pulse rate of 58/minute. His abdomen was tender with right-sided pain upon palpation. [njmonline.nl]

    Onset of the disorder is nonspecific and shows signs such as: Rigors Fever Headache Perspiration Diarrhea Vomiting Malaise Chilly sensations Abdominal pain Muscular pains The pulse rate is accelerated throughout the condition. [primehealthchannel.com]

Cardiovascular

  • Hypotension

    He was hypotensive, hyperkalemic and hyponatremic with a purpuric rash. His blood cultures grew Escherichia coli. He failed to respond to treatment, and died soon after admission due to septic shock. [candent.ca]

    As a combined result of 1º adrenal failure and massive hemorrhage, patients display hypotension, which can rapidly progress to septic shock. [picmonic.com]

    It is characterized by rapidly developing ADRENAL INSUFFICIENCY; HYPOTENSION; and widespread cutaneous PURPURA. [profiles.umassmed.edu]

    A previously healthy 19-year-old woman had a febrile illness with hypotension, progressive cyanosis, and an evolving petechial rash. [ncbi.nlm.nih.gov]

  • Cyanosis

    Waterhouse-Friderichsen syndrome - a condition characterized by vomiting, diarrhea, extensive purpura, cyanosis, tonic-clonic convulsions, and circulatory collapse. [medical-dictionary.thefreedictionary.com]

    A previously healthy 19-year-old woman had a febrile illness with hypotension, progressive cyanosis, and an evolving petechial rash. [ncbi.nlm.nih.gov]

    Asphyxia – 2. severe cyanosis [eje.bioscientifica.com]

    Furthermore, cyanosis of the whole body was evident, and purple spots were apparent on the patient’s neck and face. [hindawi.com]

Skin

  • Purpura

    Waterhouse-Friderichsen syndrome without purpura due to Haemophilus influenzae group B. Abstract A second recorded case of Waterhouse-Friderichsen syndrome, without purpura, due to Haemophilus influenzae is described. [doi.org]

    A second recorded case of Waterhouse-Friderichsen syndrome, without purpura, due to Haemophilus influenzae is described. [ncbi.nlm.nih.gov]

    Waterhouse-Friderichsen syndrome - a condition characterized by vomiting, diarrhea, extensive purpura, cyanosis, tonic-clonic convulsions, and circulatory collapse. [medical-dictionary.thefreedictionary.com]

  • Petechiae

    When a patient presents with fever and petechiae, WFS must be considered, even when the patient has a non-toxic appearance. [ncbi.nlm.nih.gov]

    Case report: We present the case of a 4 months boy with high fever 40 °C, vomiting, diarrhea, lethargy, maculopapular rash followed by petechiae and purpura. Biological tests revealed important leukocytosis and thrombocytopenia. [endocrine-abstracts.org]

    Definitions of Waterhouse-Friderichsen syndrome noun syndrome associated with bacterial meningitis; marked by sudden high fever and skin discoloration and petechiae with hemorrhage into the adrenal glands and cardiovascular collapse EDITOR’S CHOICE Test [vocabulary.com]

    {Noun} syndrome associated with bacterial meningitis; marked by sudden high fever and skin discoloration and petechiae with hemorrhage into the adrenal glands and cardiovascular collapse Add Meaning Waterhouse-Friderichsen syndrome Translate not found [howtopronounce.com]

  • Skin Rash

    Patients present with rapidly progressive septic shock, diffuse purpuric skin rash and adrenal insufficiency crisis. Treatment includes supportive therapy, intravenous antibiotics directed against the cultured organisms, and steroid therapy. [candent.ca]

    He had undergone a laparotomy following abdominal trauma at age 14 years, with splenectomy, and had a history of rheumatoid arthritis treated with methotrexate.  At necropsy a skin rash was noted. [slideshare.net]

    He had undergone a laparotomy following abdominal trauma at age 14 years, with splenectomy, and had a history of rheumatoid arthritis treated with methotrexate. At necropsy a skin rash was noted. [fr.slideshare.net]

    At necropsy a skin rash was noted. The lungs were congested and massive bilateral adrenal haemorrhages were present (fig 1). The spleen was absent and the upper peritoneum was studded with multiple soft splenunculi. [ncbi.nlm.nih.gov]

    A characteristic skin rash with a typical evolution occurs in approximately 75% of patients with Waterhouse-Friderichsen syndrome. In its early stages, the rash consists of small, pink macules or papules. [rareguru.com]

  • Skin Lesion

    Image Read Article Now Book Mark Article Skin biopsy and Gram stain A skin lesion biopsy is when a small amount of skin is removed so it can be examined. The skin is tested to look for skin conditions or diseases. A... [bmhsc.org]

    The vesicular skin lesions resolved completely after treatment. [njmonline.nl]

    Patients who appear in the initial and nontoxic-appearing stage without any skin lesions may be difficult to distinguish from a benign viral illness. [ncbi.nlm.nih.gov]

    The gram stain of the skin lesion showing gram-positive cocci likely represents contamination with normal skin flora. In 1948, Hoyne and Brown reported good sensitivity with gram-staining of skin lesions on presumably profoundly ill patients (3). [path.upmc.edu]

Urogenital

  • Anuria

    Other features may include dehydration, vomiting, diarrhoea, oliguria, neck stiffness, and occasionally anuria. Occur usually in infants or children, occasionally in adults. [whonamedit.com]

    Other clinical features may include dehydration, vomiting, diarrhea, oliguria, and neck stiffness, and occasionally anuria. Usually occurs in infants or children, ... [accesspediatrics.mhmedical.com]

    Other clinical features may include dehydration, vomiting, diarrhea, oliguria, and neck stiffness, and occasionally anuria. Usually occurs in infants or children, occasionally in adults. Death usually occurs after a few hours if not treated. [accessanesthesiology.mhmedical.com]

    Upon arrival even with persistent hypotension despite double scheme amines (dopamine, norepinephrine) and fluid resuscitation, also high ventilatory requirements with data from acute lung injury, in frank anuria and acute renal injury, severe metabolic [omicsonline.org]

  • Oliguria

    Other features may include dehydration, vomiting, diarrhoea, oliguria, neck stiffness, and occasionally anuria. Occur usually in infants or children, occasionally in adults. [whonamedit.com]

    Other clinical features may include dehydration, vomiting, diarrhea, oliguria, and neck stiffness, and occasionally anuria. Usually occurs in infants or children, ... [accesspediatrics.mhmedical.com]

    Other clinical features may include dehydration, vomiting, diarrhea, oliguria, and neck stiffness, and occasionally anuria. Usually occurs in infants or children, occasionally in adults. Death usually occurs after a few hours if not treated. [accessanesthesiology.mhmedical.com]

    Based on data gathered from clinics and laboratories, the following signs have been decided to be diagnostically significant: Albuminuria Facial edema Severe shock Marked oliguria Marked leukocytosis Hematuria or cylindruria Elevation of blood sodium [primehealthchannel.com]

Neurologic

  • Meningism

    […] including WFS, and (7) review when meningitis prophylaxis is given. [jetem.org]

    Waterhouse-Friderichsen syndrome [ waht´er-hows frid″er-ik´sen ] the malignant or fulminating form of meningococcal meningitis, which is marked by sudden onset and short course, fever, coma, collapse, cyanosis, hemorrhages from the skin and mucous membranes [medical-dictionary.thefreedictionary.com]

    Autopsy showed Haemophilus influenzae (type B) meningitis, multiple organ involvement with DIC, and bilateral adrenal hemorrhagic necrosis (Waterhouse-Friderichsen syndrome). [ncbi.nlm.nih.gov]

    Asia has been the focus of meningococcal meningitis. [e-ijd.org]

  • Headache

    Symptoms include: Fever and chills Joint and muscle pain Headache Vomiting Infection with bacteria causes bleeding throughout the body, which causes: Bodywide rash Disseminated intravascular coagulation in which small blood clots cut off blood supply [nlm.nih.gov]

    Symptoms include: Fever and chills Joint and muscle pain Headache Vomiting Infection with bacteria causes bleeding throughout the body, which causes: A rash throughout the body Disseminated intravascular coagulation in which small blood clots cut off [medlineplus.gov]

    The initial presenting complaints for patients with the WFS usually include a diversity of nonspecific, vague symptoms such as cough, dizziness, headache, sore throat, chills, rigors, weakness, malaise, restlessness, apprehension, myalgias, arthralgias [ncbi.nlm.nih.gov]

  • Seizure

    Enter a room trauma shock seizures witnessed at home and during transportation to the hospital, clouding, state initial shock, hypoglycemia, fever, with a history of non-specific prodrome 7 days prior characterized by fatigue and weakness, adding fever [omicsonline.org]

    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. [altmeyers.org]

    Patients may also present with neurological deficits, altered mental status, and seizures, indicating increased intracranial pressure (ICP). The course of viral meningitis is generally much milder. [amboss.com]

    More than 75% of patients develop a generalized rash and, if no treatment is administered, patients may present vomiting, seizures, mental status changes, violent chills, circulatory collapse, and shock.[1] Concerning the laboratory examinations, leukocytosis [journals.lww.com]

Workup

Diagnostic workup is broad, although the increasing incidence of the condition has prompted the establishment of more uniform criteria to assure proper diagnosis. The three cardinal criteria are defined by positive blood cultures for meningococci or the occasional isolation of the organisms from peripheral blood smears, specific gravity fixation on urinalysis and renal failure demonstrated by the elevation of creatinine serum levels and blood urea nitrogen [12]. Other important diagnostic markers are the following: severe shock, hypernatremia, significant oliguria, anuria for 24 to 36 hours, albuminuria, hypokalemia,  hematuria or cylindruria, significant leukocytosis, petechial rash developing into a purpuric rash and facial swelling.

Diagnosis of WFS is not limited to any particular factor of those mentioned above. In acute, rapidly progressing cases, clinical signs are sufficient to diagnose the disease. Suspected chronic cases necessitate the consideration of additional factors for conclusive diagnosis, such as lab studies and the culture or isolation of meningococcal organisms. 

Treatment

WFS is an emergency condition and antibiotics need to be promptly administered. The most common antibiotic used today is ceftriaxone. Reversal of adrenal shock can sometimes be established with hydrocortisone, and widespread necrosis may necessitate plastic surgery or grafting [13].

Treatment of WFS is directed primarily to the resolution of shock, toxemia, bacteremia and adrenal insufficiency due to decreased adrenocortical hormone levels. The treatment of septic shock follows the usual process with cautious administration of 3000 to 4000 cc of parenteral fluids over a 24 hour period. Repeated administration of 500 cc of plasma every 12 hours is also recommended due to its osmotic effect and the subsequent elevation in blood pressure. 

Toxemia results from the bacterial products of invasion and is majorly responsible for the pathophysiologic changes associated with the disease. It is generally countered with serotherapy, defined as the injection of antitoxin or a serum containing antibodies. Current treatment guidelines recommend the administration of 60,000 to 120,000 units of antimeningococcal serum in the initial 24 hours, although there is no clear evidence for its beneficial effects.

Chemotherapy has proven very useful in the control of meningococcemia. Sulfadiazine, a sulfonamide drug, is particularly effective. High parentral and oral doses are required to combat the massive bacteremia present in the disease. Initial doses of sulfadiazine consist of 5 gm injected parenterally and 8 gm taken orally, followed by large doses so that a total of 25 to 30 gm are given in the first 24 hours. Blood levels of sulfadiazine need to be monitored to adjust the dosage, with the aim of maintaining a blood level of 15 to 20 gm per 100 cc.

Prognosis

Bilateral adrenal hemorrhage carries a case fatality rate of 15% and prognosis is dependent on the nature of the underlying disease and its severity. In particular, Waterhouse-Friderichsen syndrome displays a mortality rate of 55% - 60% and prognosis worsens with any delay in diagnosis or treatment. Death in WFS is generally result of sepsis and occurs even after therapeutic or supportive treatment with antibiotics and glucocorticoid administration. Patients who later suffer chronic adrenal failure due to hemorrhage can recover, although such instances remain rare [8].

Etiology

Waterhouse-Friderichsen syndrome (WFS) results from a severe bacterial infection and is most commonly associated with a Neisseria meningitidis blood infection, the organism usually related to the occurrence of meningitis in adults and elderly populations. Other known bacterial causative agents include Pseudomonas aeruginosa, Streptococcus pneumoniae, Staphylococcus aureus, Mycobacterium tuberculosis and various gram-negative organisms such as Pasteurella spp., Klebsiella spp., Escherichia coli and Haeomophilus influenzae  [3] [4]. The variant associated with tuberculosis is caused by tubercular hemorrhagic destruction of the adrenal glands and is particularly characterized by mineralocorticoid deficiency. WFS, however, is not exclusively caused by bacterial pathogens and can sometimes result from the intake of medications that promote blood clotting, such as steroids. Other disturbances, such as low platelet counts, primary antiphospholipid syndrome and renal vein thrombosis [5] can also cause WFS.

Meningococcal WFS is highly contagious when close contact is established between an affected and a non-affected individual. Contamination usually occurs after kissing an affected individual or through inhalation of large respiratory droplets.

Epidemiology

Waterhouse-Friderichsen syndrome is a rare condition that targets mostly children younger than 10 years old. The overall prevalence of adrenal hemorrhage is much more elevated and has been estimated to be around 15% in individuals who die of shock.

Patients affected by WFS are at a high risk of death due to, either the bacterial septicemia itself, or the complications of the adrenal hemorrhage, even after treatment with high stress doses of glucocorticoids. Mortality rate amongst the patients with adrenal hemorrhage is estimated to be around 15% and is dependent on the underlying disease. It can amount to up to 55% - 60% in patients with WFS [6].

Pathophysiology

The sequence of events responsible for non-traumatic adrenal bleeding is still poorly understood, although current knowledge involves several factors that include the anatomy of the venous drainage of the adrenals (limited to a single draining vein), adrenal vein spasm, thrombosis and adrenocorticotropic hormone (ACTH) secretion related to a stress reaction.

Stress plays a central role in the pathophysiological process and it is thought that increased stress levels associated with any cause, but particularly infection, lead to an increase in ACTH secretion. This ultimately results in elevated blood perfusion to the adrenals, overwhelming the limited drainage capacity of the gland and leading to extensive hemorrhage. Stress also causes a release of catecholamines that results in spasms of the adrenal vein and subsequent venous stasis and hemorrhage. Venous stasis is exacerbated by coagulopathies that lead to thrombosis and may be directly caused by sepsis, primary antiphospholipid syndrome, disseminated intravascular coagulation (DIC) or heparin-induced thrombocytopenia [7].

Bilateral adrenal hemorrhage, regardless of the underlying process, leads to acute adrenal failure which can cause an adrenal crisis if not recognized early and treated adequately.

Prevention

Vaccination against meningococcus is the primary preventive strategy against Waterhouse-Friderichsen syndrome. Recommendations from the Center for Disease Control include routine vaccinations for all children and adolescents between 11 and 18 years of age as well as for individuals with certain immunodeficiencies or poor spleen function.

Summary

Waterhouse-Friderichsen Syndrome (WFS) is a rare disease targeting mostly children younger than 10 years old and manifests with acute adrenal failure, following hemorrhage into the adrenals and bacterial infection. Meningococcal infection is the most common cause, but other bacterial organisms are also involved. Presentation is characterized initially by widespread non-specific symptoms, although an elevated pruritic rash is very characteristic of the disease. Progression may lead to an acute adrenal crisis, shock and death, if adequate treatment is not provided [1] [2]. Diagnosis can be established clinically in acute cases, while chronic cases may necessitate the performance of a range of laboratory tests that demonstrate metabolic and hematologic abnormalities, such as hypoglycemia, hyponatremia, hyperkalemia, negative ACTH stimulation test, thrombocytopenia and hematologic changes indicative of diffuse intravascular coagulation. Ceftriaxone is the antibiotic of choice and needs to be administered promptly along with fluids and osmotic solutions to combat shock. Prognosis is poor, with mortality rates of up to 60%.

Patient Information

Waterhouse-Friderichsen syndrome (WFS) is a condition invloving extensive bleeding into the adrenal glands, which results in their functional failure. Bacterial infections are primarily responsible, particularly Nisseria meningitidis, although other organisms such as Group B streptococci, Pseudomonas aeruginosa, Streptococcus pneumoniae and Staphylococcus aureus have also been incriminated. Rare causes of WFS by factors that promote or inhibit blood clotting include medications, steroid use, low platelets, renal vein thrombosis and primary antiphospholipid syndrome.

Initial symptoms of WFS are fluctuating and non-specific. They include headaches, chills, weakness, malaise, restlessness, fever, joint pain, muscle pain, cough, dizziness and rigors. The symptoms can be very mild and non-troublesome to the patient, leading to a delayed diagnosis and ultimately a higher risk of death. A very characteristic feature of the disease is the pruritic rash caused by septicemia, which occurs in 50 to 75% of the patients. The development of a full blown blood infection and organ failure results in extensive hemorrhage into the skin, shock, confusion, coma, hypotension and ultimately death. Death can occur very rapidly, in a few hours after the onset of septicemia and the resulting hemorrhage into the adrenal glands.

Diagnosis of WFS may be established clinically in case it is acute but chronic variants are also dependent on characteristic laboratory results. Metabolic abnormalities include hypoglycemia, hyponatremia, hyperkalemia, acidosis and acute renal failure in severe sepsis. An increased number of white blood cells or a very diminished number of those can be present, alongside decreased platelets. Meningococcal organisms can be cultured from blood samples or detected from skin lesions.

WFS is an emergency and prompt administration of antibiotics is critical. The antibiotic of choice is ceftriaxone. Administration of fluids and plasma are also important to combat shock. The Center for Disease Control recommends vaccination against meningoccoci as the primary method of prevention.

References

  1. Rao RH. Bilateral massive adrenal hemorrhage. Med Clin North Am. 1995; 79(1):107-29.
  2. Vella A, Nippoldt TB, Morris JC 3rd. Adrenal hemorrhage: a 25-year experience at the Mayo Clinic. Mayo Clin Proc. 2001 Feb; 76(2):161-8.
  3. Piccioli A, Chini G, Mannelli M, et al. Bilateral massive adrenal hemorrhage due to sepsis: report of two cases. J Endochrinol Invest 1994; 17:821–4.
  4. Adem PV, Montgomery CP, Husain AN, et al. Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children. The New England journal of medicine. 2005 Sep 22; 353(12):1245-51.
  5. Arnason JA, Graziano FM. Adrenal insufficiency in the antiphospholipid antibody syndrome. Semin Arthritis Rheum. 1995 Oct; 25(2):109-16.
  6. Tormos LM, Schandl CA. The Significance of Adrenal Hemorrhage: Undiagnosed Waterhouse-Friderichsen Syndrome, A Case Series. J Forensic Sci. 2013 Mar 4.
  7. Ketha S, Smithedajkul P, Vella A, Pruthi R, Wysokinski W, McBane R. Adrenal haemorrhage due to heparin-induced thrombocytopenia. Thromb Haemost. 2013 Feb 7; 109(4).
  8. Jahangir-Hekmat M, Taylor HC, Levin H, et al. Adrenal insufficiency attributable to adrenal hemorrhage: long-term follow-up with reference to glucocorticoid and mineralocorticoid function and replacement. Endocr Pract. 2004 Jan-Feb; 10(1):55-61.
  9. Ip M, Teo JG, Cheng AF. Waterhouse-Friderichsen syndrome complicating primary biliary sepsis due to Pasteurella multocida in a patient with cirrhosis. J Clin Pathol. 1995 Aug; 48(8):775-7.
  10. Carey T. Adrenal hemorrhage with purpura and specticemia (Waterhouse Friderichsen syndrome). Ann Intern Med. 1940; 13(9):1740-1744.
  11. Friderichsen C. Waterhouse–Friderichsen syndrome (W.–F. S.). Acta Endocrinol1955; 18:482–92.
  12. Rucks W, and Hobson J. Purpura fulminans (Waterhouse-Friderichsen syndrome): report of case with recovery. J Pediat. 1943; 22:226–232.
  13. Nelson J, Goldstein N. Nature of Waterhouse-Friderichsen syndrome; report of case with successful treatment with cortisone. J Am Med Assoc. 1951 Jul 28; 146(13):1193–1197.
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