Acute generalized exanthematous pustulosis

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1Active trials16Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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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
Increased total neutrophil countHP:0011897Eosinophilic dermal infiltrationHP:0032022Predominantly dermal neutrophilic infiltrateHP:0031236AcantholysisHP:0100792CheilitisHP:0100825Skin vesicleHP:0200037
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Sep 2018Drug Reactions Sampling (COLLECTIONTOXIDERMIES)

Assistance Publique - Hôpitaux de Paris

TrialRECRUITING

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 →

Clinical Trials

1 recruitingView all trials with filters →
Other1 trial
Drug Reactions Sampling (COLLECTIONTOXIDERMIES)
Actively Recruiting
PI: Saskia Oro, MD (APHP) · Sites: Créteil · Age: 1899 yrs

Specialists

16 foundView all specialists →
RP
Rose Parisi
SMITHTOWN, NY
Specialist
1 Acute generalized exanthematous pustulosis publication
BM
Beda Muehleisen
Specialist
1 Acute generalized exanthematous pustulosis publication
MZ
Michael Ziv
Specialist
1 Acute generalized exanthematous pustulosis publication
NS
Neil H Shear
Specialist
1 Acute generalized exanthematous pustulosis publication
RD
Roni P Dodiuk-Gad
Specialist
1 Acute generalized exanthematous pustulosis publication
FT
F Tetart
Specialist
1 Acute generalized exanthematous pustulosis publication
JM
Julia-Tatjana Maul
Specialist
3 Acute generalized exanthematous pustulosis publications
BM
Barbara Meier-Schiesser
Specialist
2 Acute generalized exanthematous pustulosis publications
CW
Chunjiang Wang
Specialist
2 Acute generalized exanthematous pustulosis publications
AC
Antonio Costanzo
Specialist
2 Acute generalized exanthematous pustulosis publications
HS
Hemali Shah
Specialist
1 Acute generalized exanthematous pustulosis publication
AP
Antonio Martorell, PhD
Specialist
PI on 3 active trials
SW
S Walsh
Specialist
1 Acute generalized exanthematous pustulosis publication
BM
B Milpied
Specialist
1 Acute generalized exanthematous pustulosis publication
KG
K Gaspar
Specialist
1 Acute generalized exanthematous pustulosis publication
SI
S Ingen-Housz-Oro
Specialist
2 Acute generalized exanthematous pustulosis 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 Acute generalized exanthematous pustulosis.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Acute generalized exanthematous pustulosis

1 articles
ResearchCLINICALTRIALSMar 26, 2026
Trial Completed: Evaluating Legit.Health Plus Support for Improving Diagnosis of Generalized Pustular Psoriasis and Other Skin Conditions Among Primary Care Physicians and Dermatologists (NCT07428915)
Researchers completed a study testing whether an AI tool called Legit.Health Plus can help doctors better diagnose rare and complicated skin conditions. The too
See all news about Acute generalized exanthematous pustulosis

Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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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.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.