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

Abstract

Neisseria meningitidis-induced acute systemic meningococcal disease is an emergency and a fatal condition that has a high mortality rate. In patients with a fulminant infection, a maculopapular petechial eruption, purpura fulminans, or an ecchymotic lesion are worrisome signs reflecting disseminated intravascular coagulation (DIC) and hint at Waterhouse-Friderichsen syndrome (WFS). Here, we describe a rare case of a patient with a fulminant Neisseria meningitidis-induced acute systemic meningococcal disease presenting with high-grade fever without meningitis symptoms. Fatal septicemia with DIC and multiple organ failure was noted. WFS was chiefly suspected. We highlight the clinical features and pathogenesis of Neisseria meningitidis-induced meningococcemia and WFS. We propose that they should be kept in mind, especially in patients presenting with a petechial eruption and purpura fulminans.

Keywords: Neisseria meningitidis; Waterhouse–Friderichsen syndrome; disseminated intravascular coagulation; meningococcemia; multiple organ failure.

Figure 1

The chest X-ray revealed mild increased bilateral lung markings without a significant pneumonia patch (A) initially (B) after one day.

Figure 2

A schematic diagram illustrating the detailed pathophysiological mechanism between septicemia and disseminated intravascular coagulation in Neisseria meningitidis infection. Adapted from Chang [14,15] and Pathan et al. [13].