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Potentially life-threatening emergency
Purpura fulminans in Adult
Other Resources UpToDate PubMed
Potentially life-threatening emergency

Purpura fulminans in Adult

Contributors: Jourdan Brandon MD, Asha Nanda MD, Philip I. Song MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Purpura fulminans (PF) is a severe, often fatal subtype of disseminated intravascular coagulation (DIC) that presents with extensive tissue thrombosis and widespread skin necrosis. It can be due to inherited protein C deficiency or acquired protein C deficiency in the setting of severe bacterial infection, commonly Neisseria meningitidis. PF occurs with a bimodal incidence with peaks at 1-3 years (predominantly in patients with inherited protein C deficiency) and a second in adolescence (due to the increased incidence of N meningitidis in this group).

PF can be categorized into 3 types: acute infectious purpura fulminans, idiopathic or acquired purpura fulminans, and neonatal purpura fulminans. Acute infectious PF is the most common type and is associated with an acute infection and DIC. This type is due to an acquired deficiency of protein C secondary to the consumption of profibrinolytic cofactors. Decreased function of protein C induces a hypercoagulable state. The most common bacterial causes are Neisseria and Streptococcus pneumoniae, and varicella is the most common viral cause. PF develops in 10%-20% of patients with meningococcal septicemia. Urosepsis and staphylococcal sepsis are further common causes. Rickettsial disease, Capnocytophaga canimorsus (from a dog bite), and malaria may also lead to PF.

Acute illness, eg, septic shock, presents with high fever and rapid deterioration leading to hypotension and end-organ dysfunction. Physical or functional asplenia and immunosuppression increase the risk of developing acute infectious PF.

Idiopathic or acquired PF develops 7-10 days following a febrile illness, typically varicella or scarlet fever. It is rare and is due to an acquired protein S deficiency state. Anti-protein C or S antibodies bind to protein C or S. Cross-reacting immunoglobulin G (IgG) autoantibodies increase protein S clearance from the circulation and are thought to trigger postinfectious PF. Diminished protein S activity results in decreased activation of protein C and a subsequent hypercoagulable state. Acquired PF is associated with a lower mortality rate than the other types of PF.

Neonatal PF manifests early in life and is secondary to hereditary or acquired deficiency of protein C, protein S, or antithrombin III. See neonatal purpura fulminans for further discussion.

Codes

ICD10CM:
D65 – Disseminated intravascular coagulation [defibrination syndrome]

SNOMEDCT:
13507004 – Purpura fulminans

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Warfarin necrosis (warfarin necrosis) – Purpura from warfarin is more prominent on fatty areas such as the breasts, buttocks, and thighs, while PF is usually more extensive.
  • Necrotizing fasciitis
  • Rocky Mountain spotted fever
  • Toxic shock syndrome
  • Thrombotic thrombocytopenic purpura
  • Calciphylaxis
  • Acute meningococcemia
  • Overanticoagulation with heparin or warfarin
  • Cocaine levamisole toxicity
  • Catastrophic Antiphospholipid antibody syndrome
  • Vasculitis
  • Superficial thrombophlebitis
  • Cryoglobulinemia
  • Granulomatosis with polyangiitis
  • Eosinophilic granulomatosis with polyangiitis

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:01/18/2023
Last Updated:02/08/2023
Copyright © 2024 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Purpura fulminans in Adult
A medical illustration showing key findings of Purpura fulminans : Fever, Gangrene, Patient appears systemically ill - toxic, Ecchymosis, HR increased, BP decreased
Clinical image of Purpura fulminans - imageId=277439. Click to open in gallery.  caption: 'Retiform, purpuric, and necrotic papules and plaques on the arm and hand.'
Retiform, purpuric, and necrotic papules and plaques on the arm and hand.
Copyright © 2024 VisualDx®. All rights reserved.