Acute generalized exanthematous pustulosis

(Redirected from AGEP)

Background

  • T-cell mediated rash with systemic features
  • >90% of cases attributable to a medication (usually an antibiotic)
  • Mortality rate of ~5%

Clinical Features

Acute generalized exanthematous pustulosis
  • Onset 1-5 days after starting causative medication
  • Rash:
    • Large areas of edematous erythema with numerous small, non-follicular pustules
    • Predominantly affects main body folds and upper trunk, but can involve face
    • NO mucous membrane involvement (in contrast to SJS/TEN)
  • Systemic findings:

Differential Diagnosis

Erythematous rash

Evaluation

Workup

  • Clinical diagnosis
  • CBC
  • BMP
  • LFTs

Diagnosis

Table of Severe Drug Rashes

Charateristic DRESS SJS/TEN AGEP Erythroderma
Image PMC3894017 JFMPC-2-83-g001.png Stevens-johnson-syndrome.jpg Acute generalized exanthematous pustulosis.png Red (burning) Skin Syndrome - Feet Collage.jpg
Onset of eruption 2-6 weeks 1-3 weeks 48 hours 1-3 weeks
Duration of eruption (weeks) Several 1-3 <1 Several
Fever +++ +++ +++ +++
Mucocutaneous features Facial edema, morbilliform eruption, pustules, exfoliative dermattiis, tense bullae, possible target lesions Bullae, atypical target lesions, mucocutaneous erosions Facial edema, pustules, tense bullae, possible target lesions, possibl emucosal involvement Erythematous plaques and edema affecting >90% of total skin surface with or without diffuse exfoliation
Lymph node enlargement +++ - + +
Neutrophils Elevated Decreased Very elevated Elevated
Eosinophils Very elevated No change Elevated Elevated
Atypical lymphocytes + - - +
Hepatitis +++ ++ ++ -
Other organ involvement Interstitial nephritis, pneumonitis, myocarditis, and thydoiditis Tubular nephritis and tracheobronical necrosis Possible Possible
Histological pattern of skin Perivascular lymphocytcic infiltrate Epidermal necrosis Subcorneal pustules Nonspecific, unless reflecting Sezary syndrome or other lymphoma
Lymph node histology Lymphoid hyperplasia - - No, unless reflecting Sezary syndrome or other malignancy
Mortality (%) 10 5-35 5 5-15

Management

  • Stop inciting agent
  • IVF- treat similar to fluid resuscitation in burns
  • Wound care, infection control

Disposition

  • Admit

See Also

External Links

References

Authors:

Ross Donaldson