Overview
Acute generalized exanthematous pustulosis, often called AGEP, is a rare and serious skin reaction that is almost always triggered by medications. It causes a sudden outbreak of many tiny, non-infectious pustules (small blisters filled with pus) on top of widespread red, swollen skin. The reaction typically begins within a few hours to a few days after starting a new medication, most commonly antibiotics like aminopenicillins and macrolides, but also other drugs such as calcium channel blockers, antimalarials, and antifungals. In rare cases, viral infections or spider bites have been reported as triggers. The condition usually starts with a high fever (often above 38°C or 100.4°F) and a burning or itching sensation on the skin, especially in the skin folds and on the face and trunk. Within hours, hundreds of tiny sterile pustules appear on a background of bright red skin. Patients may also experience swelling of the face and hands, and blood tests often show a high white blood cell count. The good news is that AGEP is usually self-limiting, meaning it resolves on its own once the offending drug is stopped. Most people recover fully within one to two weeks. Treatment is mainly supportive, focusing on stopping the triggering medication, managing fever, and caring for the skin with moisturizers or mild topical corticosteroids. Severe cases may require hospitalization, especially in elderly patients or those with other health conditions. While AGEP can look alarming, the overall prognosis is very good, with a mortality rate estimated at less than 5%, mostly in patients who have significant underlying health problems.
Also known as:
Key symptoms:
Sudden high feverWidespread skin rednessHundreds of tiny pus-filled bumps on the skinBurning or itching sensation on the skinSkin swelling, especially on the faceSkin peeling as pustules resolveFeeling generally unwell or fatiguedSwollen lymph nodesJoint pain in some casesSkin tendernessElevated white blood cell countPossible mild liver or kidney involvement in severe cases
Clinical phenotype terms (23)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventAssistance Publique - Hôpitaux de Paris
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Acute generalized exanthematous pustulosis.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Acute generalized exanthematous pustulosis.
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Questions for your doctor
Bring these to your next appointment
- Q1.Which specific medication most likely caused my AGEP reaction?,Are there related medications I should also avoid in the future?,Should I undergo patch testing to confirm the triggering drug?,How should I care for my skin during the healing process?,What warning signs should prompt me to seek emergency care?,Should I wear a medical alert bracelet or carry a drug allergy card?,Is there any risk that my family members could have a similar reaction to this medication?
Common questions about Acute generalized exanthematous pustulosis
What is Acute generalized exanthematous pustulosis?
Acute generalized exanthematous pustulosis, often called AGEP, is a rare and serious skin reaction that is almost always triggered by medications. It causes a sudden outbreak of many tiny, non-infectious pustules (small blisters filled with pus) on top of widespread red, swollen skin. The reaction typically begins within a few hours to a few days after starting a new medication, most commonly antibiotics like aminopenicillins and macrolides, but also other drugs such as calcium channel blockers, antimalarials, and antifungals. In rare cases, viral infections or spider bites have been reported
How is Acute generalized exanthematous pustulosis inherited?
Acute generalized exanthematous pustulosis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Acute generalized exanthematous pustulosis typically begin?
Typical onset of Acute generalized exanthematous pustulosis is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Acute generalized exanthematous pustulosis?
Yes — 1 recruiting clinical trial is currently listed for Acute generalized exanthematous pustulosis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Acute generalized exanthematous pustulosis?
16 specialists and care centers treating Acute generalized exanthematous pustulosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.