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DermatologyEmergency

Acute Generalized Exanthematous Pustulosis (AGEP)

Acute Generalized Exanthematous Pustulosis (AGEP): Suspected AGEP → Recognize Clinical Features → STOP Causative Drug → Differential Diagnosis → Apply E...

Pathway Overview

13 steps

Algorithm Steps

13 total

  1. 01Start

    Suspected AGEP

    Acute onset of numerous small non-follicular pustules on erythematous base

  2. 02Action

    Recognize Clinical Features

    Characteristic AGEP presentation

    • Dozens to hundreds of small (<5mm) sterile pustules
    • Non-follicular distribution
    • Arising on widespread edematous erythema
    • Facial/intertriginous areas often first affected
    • Fever >38°C common
    • Burning/pruritus
    • Onset: 1-11 days after drug (usually <5 days)
  3. 03Action

    STOP Causative Drug

    Identify and discontinue culprit medication

    • Most common: Aminopenicillins, quinolones, sulfonamides
    • Pristinamycin, hydroxychloroquine
    • Terbinafine, diltiazem
    • Macrolides, cephalosporins
    • Timeline: Usually within 48h of first dose (sensitized)
    • Or up to 11 days (first exposure)
  4. 04Decision

    Differential Diagnosis

    Distinguish from other pustular conditions

    • Pustular psoriasis: History of psoriasis, slower evolution
    • Generalized pustular psoriasis: Recurrent, psoriasis history
    • Subcorneal pustular dermatosis: Chronic course
    • Bacterial folliculitis: Follicular pustules
    • DRESS: Fewer pustules, more organ involvement
  5. 05Action

    Apply EuroSCAR Validation Score

    Standardized diagnostic scoring

    • MORPHOLOGY: Typical pustules (+2), compatible (+1), insufficient (0)
    • DISTRIBUTION: Typical (+2), compatible (+1)
    • FEVER ≥38°C: Yes (+1), No (0)
    • NEUTROPHILS ≥7×10⁹/L: Yes (+1), No (0)
    • COURSE: Pustules resolve <15 days (+0), not compatible (-2)
    • HISTOLOGY: Exocytosis + pustules (+3), compatible (+2), other (-10)
    • TOTAL: ≤0=No AGEP, 1-4=Possible, 5-7=Probable, 8-12=Definite
  6. 06Action

    Diagnostic Workup

    Laboratory and histologic evaluation

    • CBC: Neutrophilia >7×10⁹/L typical, eosinophilia possible
    • CMP: Usually normal, mild hepatic elevation possible
    • Skin biopsy including pustule: Subcorneal/intraepidermal pustules
    • Histology: Papillary edema, neutrophilic + eosinophilic infiltrate
    • Pustule culture: STERILE (not infected)
  7. 07Action

    Mild AGEP

    Limited extent, stable patient

    • Outpatient management possible
    • High-potency topical corticosteroids
    • Emollients for desquamation phase
    • Antipyretics for fever
    • Close follow-up
  8. 08Action

    Supportive Treatment

    Symptomatic care for all patients

    • Topical corticosteroids (potent) for inflammation
    • Antiseptic baths/cleansers
    • Emollients during desquamation phase
    • Antihistamines for pruritus
    • Antipyretics (acetaminophen) for fever
  9. 09Action

    Course and Monitoring

    AGEP is self-limiting

    • Pustules resolve spontaneously in <15 days
    • Desquamation follows (collarette scaling)
    • Complete resolution typically by 2-3 weeks
    • No scarring expected
    • Document culprit drug
  10. 10Action

    Allergy Workup (After Resolution)

    Patch testing to identify culprit

    • Perform 6 weeks to 6 months after resolution
    • Patch test sensitivity: ~58% (higher than SJS/TEN)
    • Identifies causative drug for future avoidance
    • Document results in medical record
    • Allergy card for patient
  11. 11Outcome

    Resolution

    Excellent prognosis

    • Complete recovery expected
    • Mortality <5% (rare systemic involvement)
    • No long-term sequelae
    • Recurrence if re-exposed to culprit
  12. 12Warning

    Rare Complications

    Uncommon but reported

    • Hepatic involvement (rare)
    • Renal involvement (rare)
    • Pulmonary involvement (rare)
    • Secondary bacterial infection
    • If systemic: Manage supportively + consult derm
  13. 13Action

    Moderate-Severe AGEP

    Extensive involvement or systemic symptoms

    • Admission may be required
    • Systemic corticosteroids: Prednisone 0.5-1 mg/kg/day
    • Monitor for rare internal organ involvement
    • Fluid/electrolyte support if extensive skin loss
    • Secondary infection surveillance
  14. Path rejoins step 08Shared downstream outcome

Guideline Source

EADV European Expert Consensus for AGEP Diagnosis and Management

Clinical Safety Information

Clinical Decision Support — Not a Substitute for Clinical Judgment

Individual patient factors may require deviation from these recommendations.

Known Limitations

  • EuroSCAR score requires histopathology for definitive diagnosis
  • Must differentiate from pustular psoriasis
  • Patch testing for drug identification done after resolution
  • Rare cases may have systemic involvement
  • Does not address viral/non-drug AGEP triggers

Applicable Regions

USEU
Version 1Next review: 2027-01-11

Frequently Asked Questions

What is the Acute Generalized Exanthematous Pustulosis (AGEP)?

The Acute Generalized Exanthematous Pustulosis (AGEP) is a emergency clinical algorithm for Dermatology. It provides a structured decision tree to guide clinical decision-making, based on EADV European Expert Consensus for AGEP Diagnosis and Management.

What guideline is the Acute Generalized Exanthematous Pustulosis (AGEP) based on?

This algorithm is based on EADV European Expert Consensus for AGEP Diagnosis and Management (DOI: 10.1111/jdv.20232).

What are the limitations of the Acute Generalized Exanthematous Pustulosis (AGEP)?

Known limitations include: EuroSCAR score requires histopathology for definitive diagnosis; Must differentiate from pustular psoriasis; Patch testing for drug identification done after resolution; Rare cases may have systemic involvement; Does not address viral/non-drug AGEP triggers. Individual patient factors may require deviation from these recommendations.

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