meningiococcemia (purpura fulminans)

Introduction

Purpura fulminans refers to the skin hemorrhage resulting from disseminated intravascular coagulation associated with meningococcemia. Less frequently, DIC associated with malignancy or massive trauma may produce purpura fulminans.

Etiology

• infection with Neisseria meningitidis
• individuals with complement deficiencies (C5,C6,C7,C8) may be susceptible to chronic meningococcemia

Epidemiology

• meningococcemia occurs primarily in children & young adults
• higher rates in winter & spring
• epidemic outbreaks in crowded living conditions
  • military camps
  • nurseries

Clinical manifestations

• in all age groups, earliest symptoms are nonspecific & common to many self-limited viral infections
• leg pain, abnormal skin color, or cold hands & feet occur within 5-18 hours of initial symptoms in 72%
• skin manifestations
  • early manifestations
    • retiform pupura
    • petechial lesions especially on trunk & extremities
    • these evolve into palpable purpura with gray necrotic centers
  • late findings
    • large ecchymotic areas, especially on extremities, face & genitalia
    • these become necrotic & may require debridement
  • other lesions
    • urticaria
    • vesicles
    • maculopapules
• general manifestations
  • myalgia
  • headache
  • meningismus
  • altered sensorium
  • shock
  • sepsis more common in first few hours than meningismus
• chronic meningococcemia
  • recurrent skin eruptions
    • pink maculopapular lesions
    • nodular lesions generally on lower extremities
    • petechial lesions sometimes developing vesicular centers
    • purpuric areas with pale blue-gray centers
  • fevers, sometimes intermittent
  • arthralgias, myalgias, headaches

* images

Complications

• adrenal insufficiency
• bilateral adrenal necrosis -> Addisonian crisis is potentially fatal

Management

• treat shock with fluids & vasopressors
• pan-culture & begin antibiotics
  • 3rd generation cephalosporin
• hydrocortisone for Addisonian crisis from adrenal hemorrhage/necrosis

More general terms

• bacteremia

More specific terms

• Waterhouse-Friderichsen syndrome

Additional terms

• meningococcal polysaccharide vaccine (Menomune-A/C/Y/W-135, MenACWY-CRM, Menactra, MenACYW-D)
• Neisseria meningitidis (meningococcus)