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%
• early manifestations, general
  • fever, hypotension, & rash
  • rash may be preceded by cutaneous pain, followed by the development of petechial lesions that coalesce, forming ecchymoses
• 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

Laboratory

• microangiopathic hemolytic anemia
  • disseminated intravascular coagulation

Complications

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

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

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