Acquired purpura fulminans

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Overview

Acquired purpura fulminans is a rare and life-threatening blood clotting disorder. Unlike some forms of purpura fulminans that are present from birth, the acquired form develops during a person's lifetime, usually triggered by a severe infection or, less commonly, by the body's immune system attacking itself after an illness. The condition causes tiny blood vessels throughout the body to form dangerous clots, which at the same time leads to uncontrolled bleeding. This combination — clotting and bleeding happening at the same time — is called disseminated intravascular coagulation, or DIC. The most visible sign of acquired purpura fulminans is a distinctive skin rash made up of dark, purple-red blotches that can spread quickly and may turn into open wounds or areas of dead tissue. These skin changes happen because blood flow is being cut off to the skin. The condition can also damage internal organs, including the kidneys, lungs, and liver. In severe cases, it can lead to the loss of fingers, toes, or limbs due to tissue death. Treatment focuses on addressing the underlying cause — most often a bacterial infection such as meningococcal or pneumococcal sepsis — while supporting the body's clotting system. This may include antibiotics, blood products, anticoagulants like heparin, and intensive care support. Protein C concentrate or fresh frozen plasma may be used to restore natural clotting balance. Early recognition and aggressive treatment in an intensive care unit give the best chance of survival and reducing long-term complications.

Key symptoms:

Rapidly spreading dark purple or red blotches on the skinSkin that turns black and dies in affected areasFever and signs of serious infection or sepsisLow blood pressure and rapid heart rateBleeding from multiple sites, such as the gums, nose, or IV linesPain, swelling, or discoloration in the hands, feet, or limbsConfusion or altered consciousnessKidney failure or reduced urine outputDifficulty breathingCold or blue-tinged fingers and toesOpen sores or blisters forming over the purple skin patches

Clinical phenotype terms (25)— hover any for plain English
AcrocyanosisHP:0001063Immune dysregulationHP:0002958Reduced protein S activityHP:0004855Disseminated intravascular coagulationHP:0005521Reduced protein C activityHP:0005543HypofibrinogenemiaHP:0011900Decreased erythrocyte sedimentation rateHP:0025022Erythematous maculeHP:0025475ShockHP:0031273Macular purpuraHP:0031365
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Acquired purpura fulminans.

View clinical trials →

No actively recruiting trials found for Acquired purpura fulminans at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Acquired purpura fulminans community →

Specialists

9 foundView all specialists →
JB
Justine Brodard
Specialist
1 Acquired purpura fulminans publication
JH
Johanna A Kremer Hovinga
Specialist
1 Acquired purpura fulminans publication
JR
Jochen Karl Rössler
Specialist
1 Acquired purpura fulminans publication
MK
Mutlu Kartal-Kaess
Specialist
1 Acquired purpura fulminans publication
SL
Sophia Liniger
Specialist
1 Acquired purpura fulminans publication
TD
Tobias Dantonello
Specialist
1 Acquired purpura fulminans publication
MD
Miriam Diepold
Specialist
1 Acquired purpura fulminans publication
CA
Christoph Aebi
Specialist
1 Acquired purpura fulminans publication
AT
Alexandre THERON
Specialist
PI on 3 active trials11 Acquired purpura fulminans publications

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Acquired purpura fulminans.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Acquired purpura fulminans

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What caused my purpura fulminans, and has the underlying infection or trigger been fully treated?,What are the chances that I will need amputation, and how will that decision be made?,What long-term organ damage should I be monitored for, and how often do I need follow-up tests?,Is protein C concentrate or any other specific treatment appropriate for my case?,What vaccinations should I and my family members receive to reduce the risk of this happening again?,What rehabilitation services are available to help me recover physically and emotionally?,Are there any support groups or patient communities for survivors of purpura fulminans?

Common questions about Acquired purpura fulminans

What is Acquired purpura fulminans?

Acquired purpura fulminans is a rare and life-threatening blood clotting disorder. Unlike some forms of purpura fulminans that are present from birth, the acquired form develops during a person's lifetime, usually triggered by a severe infection or, less commonly, by the body's immune system attacking itself after an illness. The condition causes tiny blood vessels throughout the body to form dangerous clots, which at the same time leads to uncontrolled bleeding. This combination — clotting and bleeding happening at the same time — is called disseminated intravascular coagulation, or DIC. The

How is Acquired purpura fulminans inherited?

Acquired purpura fulminans follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Acquired purpura fulminans?

9 specialists and care centers treating Acquired purpura fulminans are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.