Purpura fulminans is a severe skin condition caused by hematologic abnormalities that can progress to other organs. Symptoms include painful bluish-purple areas on the skin, fever, and chills. Treatment includes antibiotics, anticoagulants, and blood transfusions, and in extreme cases, amputation may be necessary.
Purpura fulminans is a destructive skin condition caused by hematologic abnormalities that develop in the underlying blood vessels. The disorder can be acquired, inherited, or idiopathic, with no apparent cause. Purpura fulminans usually remains within the soft tissues of the limbs but can progress to other organs. The severity of the condition requires immediate diagnosis and treatment varies according to the location, extent and severity.
The symptoms of purpura fulminans may initially appear as pinpoint spots or reddened areas on the skin. These lesions rapidly evolve into painful bluish-purple areas with well-defined borders. Discoloration occurs when clots form in blood vessels beneath the surface, preventing normal blood flow. Areas can become thick and swollen. Eventually the skin turns black as the tissue dies, at which point the condition is called purpura gangrenosa.
In extreme cases, the body uses up all available clotting factors and can no longer stop the bleeding. Subcutaneous bleeding produces bruising. Purpura fulminans can cause the whole body to respond and cause fever and chills to occur in the patient. Extreme fatigue may follow, and hematology results often reveal anemia. The condition can progress to shock and death 48-72 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 chickenpox, measles, or meningitis before experiencing symptoms of purpura. The hereditary form of the affliction can involve individuals who lack the C and S proteins. These glycoproteins have anticoagulant properties and help the body in regulating clotting.
Doctors commonly prescribe antibiotic or antiviral medications if unresolved infections remain. Treatment of Purpura fulminans usually includes anticoagulants to prevent depletion of clotting factors and aid in the possible reversal of tissue necrosis. Healthcare providers can order blood thinning medications to loosen existing clots. Patients may require blood transfusions if the condition progresses to the point of hemorrhaging.
Healthcare professionals may order imaging studies to assess the extent of affected tissue and the extent of damage sustained. 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 pain management medications, although regional anesthetics are used for pain relief when larger areas of the body are affected.
Self-amputation can occur when tissue tightens and restricts blood flow. Surgeons usually remove dead or necrotic tissue. When purpura fulminans affects a large portion of a limb, including muscle and bone tissue, patients generally require amputation.
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