Acute Generalized Exanthematous Pustulosis (AGEP)
Definition: Acute Generalized Exanthematous Pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by the abrupt appearance of numerous nonfollicular, sterile pustules on an erythematous base (1).
Etiology: AGEP is commonly triggered by medications, particularly antibiotics and antifungals, but can also be associated with infections and other factors(1). It is most commonly triggered by the following drugs: pristinamycin,aminopenicillins, quinolones, hydroxychloroquine, sulfonamides, terbinafine, diltiazem, ketoconazole, and fluconazole(1,2)
Epidemiology: AGEP is a rare condition, with an estimated incidence ranging from 1 to 5 cases per million people (2).
Signs: Key signs include widespread pustular rash and fever, with the pustules favoring the trunk and intertriginous regions. Leukocytosis with an elevated neutrophil count is also a feature (1). The most frequent features in patients with systemic involvement are liver, kidney, and lung dysfunction (1,3).
Symptoms: Patients may experience fever, chills, and systemic malaise alongside the skin manifestations(3).
Differentials: Differential diagnoses include pustular psoriasis, generalized pustular exanthema, and drug-induced eruptions (1,3).
Diagnosis: Diagnosis is based on both clinical presentation and histological criteria. The EuroSCAR group created a standardized AGEP validation score that classifies patients into categories of definite, probable, possible, or no AGEP, based on morphology, clinical progression, and histology.
Treatment: Management involves discontinuation of the offending drug, supportive care, and, in severe cases, systemic corticosteroids (1,2). During the pustular phase, moist dressings and antiseptic solutions can be used to prevent infection (1).
References: (AMA)
- Szatkowski J, Schwartz RA. Acute generalized exanthematous pustulosis (AGEP): A Review and update. Journal of the American Academy of Dermatology. 2015;73(5):843-848. doi:10.1016/j.jaad.2015.07.017
- Oh DA, Yeo YW, Choo KJ, Pang SM, Oh CC, Lee HY. Acute generalized exanthematous pustulosis: Epidemiology, clinical course, and treatment outcomes of patients treated in an asian academic medical center. JAAD International. 2021;3:1-6. doi:10.1016/j.jdin.2020.12.004
- Parisi R, Shah H, Navarini AA, et al. Acute generalized exanthematous pustulosis: Clinical features, differential diagnosis, and management. American Journal of Clinical Dermatology. 2023;24(4):557-575. doi:10.1007/s40257-023-00779-3