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Concept
Acute generalized exanthematous pustulosis
Summary
Acute generalized exanthematous pustulosis (AGEP) (also known as pustular drug eruption and toxic pustuloderma) is a rare skin reaction that in 90% of cases is related to medication.
AGEP is characterized by sudden skin eruptions that appear on average five days after a medication is started. These eruptions are pustules, i.e. small red white or red elevations of the skin that contain cloudy or purulent material (pus). The skin lesions usually resolve within 1–3 days of stopping the offending medication. However, more severe cases are associated with a more persistent disorder that may be complicated by secondary skin infections and/or involvement of the liver, lung, and/or kidney.
Severe cutaneous adverse reaction (SCAR) disorders are regarded as the drug-induced activation of T cells which then initiate innate immune responses that are inappropriately directed against self tissues. Studies on the DRESS syndrome, Stevens–Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and SJS/TEN overlap indicate that many individuals are predisposed to develop these reactions to a particular medication based on their genetically determined expression of particular human leukocyte antigen (i.e. HLA) alleles or T-cell receptors and/or their efficiencies in adsorbing, distributing to tissues, metabolizing, and/or eliminating) a particular SCARS-inducing medication. Evidence for these predispositions in AGEP has not been as well-established.
AGEP is an acute drug eruption characterized by numerous small, primarily non-follicular, sterile skin pustules arising within large areas of red swollen skin usually within days of taking an inciting drug. The skin eruptions are often pruritic and accompanied by fever, headache, a high number of neutrophils and eosinophils in the blood, and elevated blood levels of markers for inflammation (i.e. erythrocyte sedimentation rate and C-reactive protein). The skin eruptions typically end within a week after causative drug is discontinued.
Rare cases of lung and bone marrow involvement have also been reported to complicate AGEP.
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