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Potentially life-threatening emergency
Purpura fulminans in Child
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Potentially life-threatening emergency

Purpura fulminans in Child

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Contributors: Sophia Delano MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Purpura fulminans (PF) refers to the cutaneous manifestations that are triggered from a coagulopathic state in the setting of infection, deficiency of anticoagulant proteins, or a combination of the two.

PF presents as areas of tenderness that manifest erythema before progressing to well-demarcated retiform necrotic plaques, often of the hands and feet. Secondary vesicles and bullae may arise. The necrosis resolves with eschar formation, and gangrene may develop.

Systemic manifestations include disseminated intravascular coagulation (DIC) and multi-system organ failure. PF has a high rate of morbidity and mortality. It may be triggered by a host of conditions including bacterial and viral infections or deficiencies in anticoagulants. In some patients with known anticoagulant deficiencies, infections can be a secondary trigger. Acute illness is manifest by a septic-appearing patient with fever and rapid deterioration leading to hypotension and shock.

Neonatal PF typically presents shortly after birth with diffuse cutaneous necrosis in patients with homozygous deficiencies in protein C or protein S. Visceral thromboses are seen in this cohort. Infantile cases of PF are also associated with underlying coagulopathies and may present at several months of age. Patients with deficiencies in factor V and prothrombin III have also developed PF. See neonatal purpura fulminans for further discussion.

Infectious etiologies for PF include Neisseria meningitidis, pneumococcus, Staphylococcus aureus, Streptococcus pyogenes, and Streptococcus agalactiae (group B Streptococcus) in neonates, as well as other less common bacterial infections. PF can also be seen after varicella and rubella infections.

Codes

ICD10CM:
D65 – Disseminated intravascular coagulation [defibrination syndrome]

SNOMEDCT:
13507004 – Purpura fulminans

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura) presents as petechial and purpuric lesions on the distal legs and buttocks with accompanying abdominal pain and arthralgias. HSP lesions may develop a necrotic, crusted center but do not develop eschars or gangrene.
  • Necrotizing fasciitis will likely have a more limited area of involvement compared with PF and occurs frequently with a history of recent trauma to the involved area. Necrotizing fasciitis may present as an area of very tender, indurated erythema and not frank necrosis.
  • Rocky Mountain spotted fever (RMSF) – Children often present with fever and petechial rash and may have abdominal pain or pharyngitis-like symptoms. Children of any age presenting with such symptoms in the spring or summer (particularly in endemic areas of North Carolina, Tennessee, Oklahoma, Arkansas, and Missouri) should be empirically started on doxycycline. Concurrent infections with group A streptococcus (GAS) is possible, and positive GAS testing does not exclude the additional diagnosis of RMSF.
  • Toxic shock syndrome (TSS) – Patients with both PF and TSS may be septic appearing and hypotensive. TSS typically presents with a more widespread erythema and not skin necrosis. Streptococcal TSS is frequently associated with recent localized trauma.
  • Thrombotic thrombocytopenic purpura (TTP) presents with scattered petechiae and purpura as platelets are consumed by diffuse microthrombi. A hemolytic anemia, altered mental status, and jaundice may develop.
  • Idiopathic thrombocytopenic purpura (ITP) patients will have a thrombocytopenia but none of the coagulopathies demonstrated by PF patients with DIC. Lesions of ITP will typically be petechial or purpuric without necrosis. ITP in children often occurs after viral infection and is typically self-limited.
  • Cryoglobulinemia – Majority of cases are seen with underlying hepatitis C infection or malignancy.
  • Calciphylaxis – PF and calciphylaxis both present with areas of tender necrosis. Calciphylaxis is typically seen in patients with renal disease.
  • Disseminated fungal infection – Immunocompromised patients may present with nonhealing necrotic lesions as a sign of fungemia.
  • Coumadin necrosis (warfarin necrosis) – The purpura from Coumadin (warfarin) is more prominent on fatty areas such as breasts, buttocks, and thighs, while PF is more extensive. Lesions often rapidly progress to purpuric bullae.
  • Overanticoagulation with heparin or Coumadin
  • Trauma

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 06/07/2017
Last Updated: 12/14/2018
Copyright © 2019 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Purpura fulminans in Child
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Purpura fulminans : Chills, Fever, Gangrene, Patient appears systemically ill - toxic, Ecchymosis, HR increased, BP decreased
Clinical image of Purpura fulminans
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