Meningococcal disease

Introduction and cause

Neisseria meningitidis, a Gram-negative diplococcus, causes meningococcal disease that manifests primarily as meningitis, meningococcemia, or combined forms, and can rapidly progress to sepsis / distributive shock. (1)

Epidemiology and transmission

Invasive meningococcal disease remains rare in the United States, with an annual incidence of 0.11 cases per 100,000 population. (1)

People at increased risk for meningococcal disease include those with asplenia, HIV, or complement deficiencies — including patients on complement inhibitors such as eculizumab, ravulizumab, pegcetacoplan, or iptacopan. (2) Meningococcal disease progresses rapidly, with a case fatality rate of 10–15%.

Transmission occurs person-to-person via respiratory droplets or close contact. Asymptomatic nasopharyngeal carriers are most common in adolescents and young adults (carriage rates 10–15%). (3)

Clinical features

The cutaneous hallmarks are acral purpura and petechiae on the lower extremities that rapidly disseminate centripetally. (1) The rash may progress to purpura fulminans, which manifests as retiform purpura with extensive dermal necrosis and hemorrhagic bullae formation.

In fulminant cases, symmetric acral gangrene may develop with epidermal necrosis, sharply demarcated violaceous mottling, and eschar formation on fingers, toes, distal extremities, and earlobes.

Complications and sequelae (3)

Up to 20% of survivors of meningococcemia have chronic long-term effects, such as deafness or gangrene requiring amputation of extremities or digits.

Massive adrenal hemorrhage (Waterhouse-Friderichsen syndrome) may present with bilateral flank ecchymoses and acute adrenal insufficiency.

Other complications include disseminated intravascular coagulation, arthritis, or permanent musculoskeletal and neurological problems. Patients with purpura fulminans may heal with extensive cutaneous scarring and dyschromia.

Testing and diagnosis (6)

• Maintain a high clinical suspicion when patients present with fever and rapidly progressive acral purpura / petechiae.

  • PCR of blood and cerebrospinal fluid (CSF) are helpful in rapid presumptive identification.

  • Gram stain showing Gram-negative diplococci is helpful in confirming the diagnosis.

• Perform a head CT prior to lumbar puncture if focal neurologic deficits suggest elevated intracranial pressure.

Patient management and treatment (3)

• Rapidly administer empiric antibiotics.

• Patients must be hospitalized immediately with ICU-level care.

  • Supportive measures address shock, coagulopathy, and tissue necrosis (consider surgical debridement).

• Administer post-exposure prophylaxis urgently (<24h after identification of index patient) to close contacts using antibiotics, such as ceftriaxone (recommended for pregnant women), ciprofloxacin, and rifampin.

Prevention (7)

Routine adolescent vaccination is recommended with quadrivalent MenACWY-D (initial vaccination at 11–12 years, booster at 16 years). MenB vaccines (Bexsero, Trumenba) are recommended for high-risk college students (16–23 years) and during outbreaks.

Vaccinate asplenic patients, complement-deficient individuals, and PLWH with boosters every 3–5 years.