Acute generalized exanthematous pustulosis: A retrospective study of 51 cases in Taiwan

Background/Objective: Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse drug reaction characterized by fever and numerous sterile non-follicular pustules. It is mainly attributed to drugs, although other factors have been implicated. The objective of this study was to evaluate the clinical and histological features of AGEP in a Taiwanese population. Methods: In this retrospective study, we reviewed patients diagnosed with AGEP with a EuroSCAR (RegiSCAR) validation score more than 4 (4, probable to definite cases), between 1992 and 2012 at the Chang Gung Memorial Hospital in Taiwan. Demographic, clinical and laboratory data, pathologic findings, and disease causality were analyzed. Results: A total of 51 patients were included in this study, with 34 (66.7%) patients being diagnosed with AGEP with drug causality, and 17 (33.3%) patients being diagnosed with AGEP without drug causality. Cases of AGEP with drug causality showed an older average age, and a significantly higher rate of previous drug hypersensitivity history compared to cases of AGEP without drug causality (p = 0.0018). None of the patients had a history of psoriasis or had developed psoriasis at the 1-year follow-up. A total of 12 cases (23.5%) had systemic involvement, including liver and kidneys. Penicillin or aminopenicillin (17.6%) and cephalosporins (17.6%) were the most common causative drug groups related to AGEP. In AGEP patients without drug causality, three cases of pathogen infections were identified (1 case of mycoplasma, Coxsackie virus, and Epstein-Barr virus, respectively). Conclusion: We found that beta-lactam antibiotics were the major drug class responsible for inducing AGEP in a Taiwanese population, but that some infectious pathogens may also contribute to AGEP development.