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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) early manifestations, general - fever, hypotension, & rash - rash may be preceded by cutaneous pain, followed by the development of petechial lesions that coalesce, forming ecchymoses 4) 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 5) general manifestations - myalgia - headache - meningismus - altered sensorium - shock - sepsis more common in first few hours than meningismus [4] 6) 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] Laboratory: - microangiopathic hemolytic anemia - disseminated intravascular coagulation Complications: - adrenal insufficiency - bilateral adrenal necrosis -> Addisonian crisis is potentially fatal [6] - disseminated intravascular coagulation 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

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

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