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meningiococcemia (purpura fulminans)

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: 1) meningococcemia occurs primarily in children & young adults 2) higher rates in winter & spring 3) epidemic outbreaks in crowded living conditions a) military camps b) nurseries Clinical manifestations: 1) in all age groups, earliest symptoms are nonspecific & common to many self-limited viral infections [3] 2) leg pain, abnormal skin color, or cold hands & feet occur within 5-18 hours of initial symptoms in 72% [3] 3) skin manifestations a) early manifestations - retiform pupura - petechial lesions especially on trunk & extremities - these evolve into palpable purpura with gray necrotic centers b) late findings - large ecchymotic areas, especially on extremities, face & genitalia - these become necrotic & may require debridement c) other lesions - urticaria - vesicles - maculopapules 4) general manifestations - myalgia - headache - meningismus - altered sensorium - shock - sepsis more common in first few hours than meningismus [4] 5) chronic meningococcemia a) 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 b) fevers, sometimes intermittent c) arthralgias, myalgias, headaches * images [5,6] Complications: - adrenal insufficiency - bilateral adrenal necrosis -> Addisonian crisis is potentially fatal [6] Management: 1) treat shock with fluids & vasopressors 2) pan-culture & begin antibiotics - 3rd generation cephalosporin 3) hydrocortisone for Addisonian crisis from adrenal hemorrhage/necrosis

Related

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

Specific

Waterhouse-Friderichsen syndrome

General

bacteremia

References

  1. H. Quinny Cheng, USSF Fresno lecture, Oct 21, 1998
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 95
  3. Thompson MJ, Ninis N, Perera R, Mayon-White R, Phillips C, Bailey L, Harnden A, Mant D, Levin M. Clinical recognition of meningococcal disease in children and adolescents. Lancet. 2006 Feb 4;367(9508):397-403. PMID: 16458763
  4. Thompson MJ et al, Clinical recognition of meningococcal disease in children and adolescents. Lancet 2006, 367:397 PMID: 16458763
  5. Kugai T, Nakagawa H. (images) Evolution of Purpura Fulminans. N Engl J Med 2017; 376:2182. June 1, 2017 PMID: 28564574 http://www.nejm.org/doi/full/10.1056/NEJMicm1700231
  6. Elkston CA, Elkston DM Bacterial Skin Infections: More Than Skin Deep. Medscape. July 19, 2021 https://reference.medscape.com/slideshow/infect-skin-6003449