Acute generalized exanthematous pustulosis (AGEP) - A clinical reaction pattern

Background: A wide range of diseases or reactions can cause pustular erupti ons of the skin. In this spectrum there seems to be a subgroup with charact eristic clinical features and a typical course which is mostly caused by dr ugs for which the term acute generalized exanthematous pustulosis (AGEP) ha s been established. Objective: To describe the clinical features of AGEP. Methods: The authors' experience from a multinational epidemiological study on severe cutaneous adverse reactions and a comprehensive review of the li terature were used to provide an overview of the disease and it's possible causes. An algorithm for validating cases which was established for this st udy is also presented. Results: AGEP typically presents with at least dozens of non follicular ste rile pustules occurring on a diffuse, edematous erythema pre dominalty in t he folds and/or on the face. Fever and elevated blood neutrophils are commo n. Histopathology typically shows spongiform subcorneal and/or intraepiderm al pustules, a marked edema of the papillary dermis, and eventually vasculi tis, eosinophils and/or focal necrosis of keratinocytes. Onset is acute, mo st often following drug intake, but viral infections can also trigger the d isease. Pustules resolve spontaneously in less than 15 days. Conclusion: The diagnosis AGEP should be considered in cases of acute pustu lar rashes and detection of the causative drug should be strived for. Knowl edge of the clinical features and usual course of this disease can often pr event unnecessary therapeutical measures.