A unique fatal case of Waterhouse–Friderichsen syndrome caused by Proteus mirabilis in an immunocompetent subject

Abstract

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.

Post-Mortem Investigations for the Diagnosis of Sepsis: A Review of Literature

“…), or identification of sepsis as cause of death in patients with infectious complications arising from different conditions (e.g., burns, immobilization, alcoholism, intravenous drug addiction, big traumas, food intoxication, etc.) [ 4 , 59 , 60 , 61 , 62 , 63 ]. It is thus fundamental to provide a complete forensic analysis of the case in order to perform a certainty diagnosis.…”

Neisseria meningitidis Induced Fatal Waterhouse–Friderichsen Syndrome in a Patient Presenting With Disseminated Intravascular Coagulation and Multiple Organ Failure

“…The typical symptoms of WFS include fever, maculopapular skin rash, petechiae, ecchymosis, cyanosis of the extremities, and shock resulting from severe sepsis via coagulopathy [9]. It could also be caused by other bacteria, such as Proteus Mirabilis, Pseudomonas aeruginosa, Streptococcus pneumoniae, Mycobacterium tuberculosis, and Haemophilus influenzae [10,11]. WFS is a catastrophic syndrome, and it is difficult to be diagnosed in the clinical course due to hypotension and quick progression to shock.…”

A Rare Case of Waterhouse-Friderichsen Syndrome Without Purpura Secondary to Haemophilus Influenzae

“…The WFS is a rare disease and mostly fatal, and it occurs acutely in most cases [3][4][5][6][7][8][9]. It is mainly diagnosed at post-mortem; thus, the need for a high index of suspicion and urgent intervention is required.…”

“…It is mainly diagnosed at post-mortem; thus, the need for a high index of suspicion and urgent intervention is required. Neisseria meningitidis is isolated in more than 80% of WFS cases, other bacteria are Haemophilus influenzae and Pseudomonas aeruginosa, as seen in this case, Streptococcus pneumoniae, Staphylococcus aureus, group A Streptococcus, Neisseria gonorrhoeae, Proteus mirabilis, Klebsiella, and Legionella [4][5][6][7][8][9][10]. It is essential to know that bilateral adrenal haemorrhage on its own has other causes besides sepsis.…”

“…It could have been worsened by direct oral anticoagulation as the patient was on apixaban and INR on admission was 3. The cause is unknown sometimes, as evidenced by documented cases of idiopathic bilateral adrenal haemorrhage [5][6][7][8][9][10][11].…”