A unique fatal case of Waterhouse-Friderichsen syndrome caused by Proteus mirabilis in an immunocompetent subject: Case report and literature analysis.

INTRODUCTION: The Waterhouse-Friderichsen syndrome (WFS), also known as purpura fulminans, is a potentially lethal condition described as acute hemorrhagic necrosis of the adrenal glands. It is often caused by infection. Classically, Neisseriae meningitidis represents the main microorganism related to WFS, although, infrequently, also other infectious agents are reported as a possible etiologic agent. The authors report the first case of death due to Proteus mirabilis infection, with postmortem evidence of WFS. PATIENT CONCERNS: After a facial trauma that provoked a wound on the nose, the subject, a healthy 40-years old man, was conducted to the local hospital (in Sicily, Italy) after the primary care he was discharged. Subsequently, after 2 days of general malaise, he returned to the hospital due to the worsening of the clinical condition. During the hospitalization, hypotension, and neurological impairment appeared; the laboratory analysis showed leukocytosis and the alteration of renal, hepatic and coagulative parameters. Microbiological blood analysis resulted positive for a P mirabilis infection. DIAGNOSIS: Multiorgan failure (MOF) with disseminated intravascular coagulation (DIC) due to sepsis was diagnosed. INTERVENTIONS: The practitioners administered intensive support, antibiotic therapy, antithrombin III, vitamin K, and plasma. OUTCOMES: After 3 days the subject died. The autopsy and the microscopic investigation were performed revealing, also, the adrenal diffuse micronodular hyperplasia associated with a cortico-medullary hemorrhagic apoplexy. CONCLUSION: To our knowledge, this is the first case of MOF with WFS due to P mirabilis infection. This case report suggests that P mirabilis should be added to the list of unusual bacteria causing WFS. Furthermore, it supports the theory that any bacterium which causes DIC may cause adrenal hemorrhage and should suggest to clinicians the importance to consider a potential adrenal involvement in every patient with sepsis and DIC.

A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster infection.

Primary Varicella zoster virus infection in adults is associated with a higher risk of complications when compared with the benign disease course of primary infection during childhood. We present a rare complication of adult primary Varicella zoster in the form of acute, irreversible adrenal insufficiency due to bilateral adrenal haemorrhage, which is also known as the WaterhouseFriderichsensyndrome.

[Dyspnea and skin rash in a 49-year-old male patient]. / Dyspnoe und Hautausschlag bei einem 49-jährigen Patienten.

We report on the case of a 49-year-old man who presented with increasing dyspnea and a skin rash. The community-acquired pneumonia was initially treated with broad spectrum antibiotics. The patient's respiratory condition rapidly worsened and the clinical picture of Waterhouse-Friderichsen syndrome developed with disseminated intravasal coagulopathy and necrosis of the toes. An infection with Capnocytophaga canimorsus, which had been caused by an initially unmentioned dog bite was confirmed. In view of the fulminant course and the high risk of operative treatment of the ubiquitous necroses in all limbs, a joint decision for deescalation of therapy was made together with relatives. The patient died 14 days after admission to hospital.

A rare case of Waterhouse-Friderichsen syndrome caused by Capnocytophaga canimorsus in an immunocompetent patient.

A 53-year-old Caucasian male with hypertension and active tobacco abuse presented to a community hospital with a 2-day history of vague abdominal pain, myalgia and increased lethargy after being bitten on his right hand by the family dog while camping just 3 days prior to symptom onset. He expired within 90 min upon arrival to our intensive care unit. Pre-mortem blood cultures grew a fastidious Gram-negative aerobic rod that was identified as Capnocytophaga canimorsus. Autopsy findings showed multi-organ disseminated intravascular coagulopathy with microthrombi along with bilateral adrenal hemorrhage and necrosis of the adrenal glands consistent with Waterhouse-Friderichsen syndrome. This case contributes to the medical literature as a rare presentation of Capnocytophaga canimorsus infection in an otherwise immunocompetent patient and stresses the importance of a thorough history taking and physical examination by clinicians along with prompt administration of appropriate antibiotics.

Muerte súbita por sepsis meningocócica: diagnóstico post mórtem / Sudden death from meningococcal sepsis: postmortem diagnosis

La presentación más común de la enfermedad meningocócica es la meningitis, con una tasa de incidencia de 0,92 por 100.000 habitantes y una letalidad del 40,3% en España. Un cuadro meníngeo puede cursar con síntomas inespecíficos como la cefalea, entre otros, de tal forma que en ocasiones progresa rápidamente en cuestión de horas y provoca la muerte del paciente antes de haber recibido una atención médica adecuada. Además de la meningitis clásica, el meningococo produce una enfermedad sistémica que incluye la sepsis meningocócica y la coagulopatía intravascular diseminada. En pacientes con sepsis meningocócica se ha descrito el síndrome de Waterhouse-Friderichsen, que se caracteriza por hemorragia suprarrenal bilateral, coagulación intravascular diseminada e hígado de shock, y que produce una bacteriemia grave. Esta combinación provoca unshock fulminante y, si no se trata, puede producir muerte súbita. Presentamos el caso de una paciente con odinofagia, fiebre, vómitos y deposiciones diarreicas de 24 horas de evolución, que a pesar de recibir asistencia médica ambulatoria y hospitalaria fallece como consecuencia de una sepsis fulminante por Neisseria meningitidis y síndrome de Waterhouse-Friderichsen no diagnosticado clínicamente (AU)

The most common presentation of meningococcal disease is meningitis, with an incidence rate of 0.92 per 100,000 inhabitants and a mortality rate of 40.3% in Spain. Meningeal disease may present with nonspecific symptoms such as headache, among others, sometimes progressing rapidly to a fatal outcome before the patient receives adequate medical care. Besides classical meningitis, Neisseria meningitis produces a systemic disease that includes meningococcal sepsis and disseminated intravascular coagulopathy. In patients with meningococcal sepsis the Waterhouse-Friderichsen syndrome is described, which is characterized by bilateral adrenal hemorrhage, intravascular coagulation and shock liver, producing a severe bacteremia. This combination causes a fulminant shock and, if untreated, it may cause sudden death. We report the case of a female patient with sore throat, fever, vomiting and diarrhea of 24 hours of evolution. Despite receiving inpatient and outpatient medical care, she died as a result of N. meningitidis fulminant sepsis and clinically undiagnosed Waterhouse-Friderichsen syndrome (AU)

Waterhouse Friderichsen syndrome complicating fulminant Enterobacter cloacae sepsis in a preterm infant: the unresolved issue of corticosteroids.

Bilateral adrenal hemorrhage can complicate severe sepsis in the neonate and is most commonly attributed to meningococcal disease; however, it can be caused by other etiologic agents as well. We report herein a fatal case of Enterobacter cloacae sepsis in a preterm infant, resulting in massive adrenal hemorrhages. This is the first documented case of adrenal hemorrhage following infection with this pathogen.

[Waterhouse-Friderichsen syndrome associated to a Morganella morganii and Enterococcus faecium peritonitis]. / Syndrome de Waterhouse-Friderichsen au cours d'une péritonite à Morganella morganii et Enterococcus faecium.

About fifty to sixty percent of patients with septic shock acquire acute adrenal insufficiency. This insufficiency is most often relative, but can sometimes be absolute. Bilateral adrenal haemorrhage is a rare aetiology of absolute acute adrenal insufficiency. It is classically described in patients with severe meningococcemia (purpura fulminans), who commonly present many of the risk factors associated with bilateral adrenal haemorrhage (shock, coagulation disorders, sepsis). We report a case of bilateral adrenal haemorrhage during a peritonitis complicated by a septic shock, with no coagulation disorder. This observation shows up that this bilateral adrenal haemorrhage can complicate severe sepsis of various origins, and not only severe meningococcemia. It can be suspected in face of a septic shock with an unfavourable evolution despite adequate treatment.

Spontaneous Waterhouse-Friderichsen syndrome in a gang-housed baboon.

Waterhouse-Friderichsen syndrome can cause acute death in the baboon without specific signs. Furthermore, this syndrome could result from stress-related intestinal permeability changes that allow macromolecules and/or microbiological entities to enter the systemic circulation. The resulting sepsis could cause adrenocortical insufficiency, hypotension and shock leading to death.

Púrpura fulminans asociada a sepsis meningocócica. Presentación de un caso en una paciente adulta / Meningococcal sepsis-associated purpura fulminans. Report of a case in an adult female

Púrpura fulminans es una rara entidad caracterizada por el desarrollo rápido de grandes áreas purpúricas con bordes geográficos, frecuentemente necróticas, asociada con hiperpirexia, hipotensión y coagulopatía de consumo. El examen histopatológico revela la presencia de trombos en los capilares y vénulas cutáneos, junto con hemorragia intersticial y diversos grados de necrosis cutánea.Presentamos un caso de púrpura fulminans asociada a sepsis meningocócica en una paciente adulta previamente sana. (AU)

[A fatal case of overwhelming postsplenectomy infection syndrome developing 10 years after splenectomy].

UNLABELLED: Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis. This syndrome is called overwhelming postsplenectomy infection (OPSI) in Europe and America. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. We treated one adult patient with OPSI syndrome that developed 10 years after splenectomy. CASE: A 26-year-old man had undergone a splenectomy following a traffic accident 10 years previously. On January 7, 1996, he had diarrhea and nausea. On January 10, he became drowsy and presented at our hospital with multiple organ failure. He underwent hemodialysis and plasmapheresis because of acute renal failure and also received immune globulin, antibiotics and prednisolone. However, these medications were not effective. He died 7 hours later. We identified diplococcus on a blood smear, IgG 3 deficiency and a low titer of specific pneumococcal IgG 2 antibody. The autopsy findings included bilateral acute hemorrhagic necrosis of the adrenal glands (Waterhouse-Friderichsen syndrome).