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Purpura fulminans is a destructive skin condition caused by hematological abnormalities that develop in the underlying blood vessels. The disorder may be acquired, inherited, or idiopathic, having no apparent cause. Purpura fulminans usually remains within the soft tissues of the limbs but can progress to other organs. The seriousness of the condition requires immediate diagnosis, and treatment varies depending on location, extent, and severity.
Symptoms of purpura fulminans may first appear as pinpoint spots or reddened areas on the skin. These lesions quickly evolve into painful bluish purple areas with well-defined borders. Discoloration occurs as clots form in the blood vessels beneath the surface, impeding normal blood flow. The areas may become thick and swollen. Eventually the skin blackens as the tissue dies, at which point the condition is called purpura gangrenosa.
In extreme cases, the body consumes all of the available clotting factors and can no longer stop the bleeding. Subdermal hemorrhaging produces bruising. Purpura fulminans may cause the entire body to respond and the patient to experience fever and chills. Extreme fatigue may follow, and hematology results often reveal anemia. The condition may progress to shock and death in 48 to 72 hours after onset.
The acquired form of the affliction typically involves a previous bacterial or viral infection. Bacteria commonly associated with the condition include Escherichia coli, Staphylococcus and Streptococcus. Pediatric patients may have had chicken pox, measles, or meningitis prior to exhibiting purpura symptoms. The inherited form of the affliction may involve individuals who lack proteins C and S. These glycoproteins have anticoagulant properties and aid the body in coagulation regulation.
Physicians commonly prescribe antibiotic or antiviral medications if unresolved infections remain. Purpura fulminans treatment usually includes anticoagulants to prevent depletion of coagulation factors and aid the possible reversal of tissue necrosis. Health care providers may order clot-busting medications to dissolve existing clots. Patients may require blood transfusions if the condition progresses to the point of hemorrhage.
Health care providers may order imaging studies in order to evaluate the extent of the affected tissue and the amount of damage incurred. Individuals often receive intravenous fluids to correct metabolic imbalances and maintain organ function. Oxygen therapy ensures that adequate oxygenation is maintained. Patients may receive topical, oral, or intravenous medications for pain management, though regional anesthetics are used to alleviate pain when larger areas of the body become affected.
Auto-amputation can occur as tissues tighten and restrict blood flow. Surgeons usually remove necrotic or dead tissue. When purpura fulminans affects a large portion of a limb, including muscle and bone tissue, patients generally require amputation.