Acute Generalized Exanthematous Pustulosis
AGEP, mostly caused by drugs, is characterized by the acute occurrence of numerous sterile pinhead-sized pustules on an oedematous erythema accompanied by fever and leucocytosis. The drugs with the highest risk to cause AGEP are antibacterial agents like
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Acute Generalized Exanthematous Pustulosis Alexis Sidoroff
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AGEP, mostly caused by drugs, is characterized by the acute occurrence of numerous sterile pinhead-sized pustules on an oedematous erythema accompanied by fever and leucocytosis. The drugs with the highest risk to cause AGEP are antibacterial agents like ampicillin/amoxicillin, and quinolones, pristinamycin, anti-infective sulfonamides, the antimycotic drug terbinafine, (hydroxy)chloroquine, and diltiazem, but a large number of other drugs as well as infections have been reported as triggers. AGEP is an acute and sometimes severe reaction, but resolves quickly and without special treatment when the culprit drug is withdrawn.
11.1 Introduction The justification for addressing acute generalized exanthematous pustulosis (AGEP) in the context of life-threatening skin diseases is not so much due to a high mortality rate, but rather explained by three circumstances. Firstly, AGEP often has a quite drastic clinical appearance which, by its acute evolution,
A. Sidoroff Department of Dermatology and Venereology, Medical University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria
suggests a very severe disease. Secondly, because of misinterpretations or misclassifications as well as real overlap-cases, AGEP is often positioned in the vicinity of much more severe diseases like Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). Only as a third point, AGEP can be the cause of death in patients with poor general condition due to old age or underlying disease. Although the term AGEP is now more and more recognized and used for a characteristic pustular reaction pattern mostly due to drug intolerance, the nomenclature in the literature has been very heterogeneous and even misleading over the years. Already in Baker and Ryan’s report of 104 cases of pustular psoriasis, five patients showed a very acute and quickly resolving pustular eruption; no previous history of psoriasis and drugs and/or infections had been suspected as trigger. The authors used the term exanthematic pustular psoriasis for this particular subgroup of patients. Other names found in the literature for similar cases were pustular drug rash [1] or toxic pustuloderma [2] and often patients sharing similar clinical features were interpreted as suffering from special variants of other pustular diseases. The term pustuloses exanthématique aiguës généralisés (PEAG) was introduced to the French literature by Beylot et al. [3]. Adopted under the acronym AGEP (acute generalized exanthematous pustulosis) in the English literature, the term is now widely used for this type of reaction, although other denominations can still be found. In this context — to prevent confusion — it is important to emphasize that the term pustulosis acuta generalisata [4–6] describes a poststreptococcal disease arising mainly in children, which is an entity totally different from AGEP.
J. Revuz et al. (eds.), Life-Threatening Dermatoses and Emergencies in Dermatology, DOI: 10.1007/978-3-54
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