Drug Triggers and Clinic of Acute Generalized Exanthematous Pustulosis (AGEP): A Literature Case Series of 297 Patients

Acute generalized exanthematous pustulosis (AGEP) is a rare skin reaction, commonly caused by drugs. Available evidence mostly relies on small studies or case reports. We collected published AGEP case reports and, subsequently, described the patient characteristics, suspect and concomitant drugs, time to onset, disease management, and clinical prognosis. This study included 297 AGEP patients (64.3% women) obtained from 250 published case reports or case series with individual patient data. AGEP affected patients of all ages, but the majority of patients (88.2%) were ≥25 years old. The most frequently reported suspect drugs were anti-infectives for systemic use (36.5%), particularly antibacterials for systemic use (31.0%), and especially beta-lactam antibacterials (18.3%) and macrolides (4.3%). Other frequent suspect drugs were antineoplastics (12.2%), and anti-inflammatory/anti-rheumatic products (5.2%) plus hydroxychloroquine (12.8%). Mean time to onset was 9.1 days (standard deviation SD 13.94). Some patients developed fever (64.3%) and systemic involvement (18.9%), and most patients (76.4%) received pharmacological treatment for AGEP. Seven patients died, although five of them were already critically ill prior to AGEP. In conclusion, antibiotics remain the most common suspected cause of AGEP. While case mortality rate may be up to 2.5%, disentangling the role of AGEP on the fatal outcome from the role of the preexisting health conditions remains challenging.

Acute localized exanthematous pustulosis: A novel side effect of piroxicam

Acute generalized exanthematous pustulosis (AGEP) is a rare yet well-known cutaneous reaction pattern, mostly caused by drugs. Acute localized exanthematous pustulosis (ALEP) is a localized variant of AGEP. A 42-year-old female presented with multiple erythematous pustules on the face, which appeared three days after the intramuscular injection of piroxicam. Histopathology revealed subcorneal pustules, epidermal spongiosis, and mixed inflammatory cell infiltration in the dermis. The pustules resolved within several days once the patient had discontinued the drug. Herein, we report the first case, as far as we know, of a female with a cutaneous drug reaction consistent with ALEP caused by piroxicam.

Case Report: Successful Treatment of Acute Generalized Exanthematous Pustulosis With Secukinumab

Acute generalized exanthematous pustulosis is a severe, usually drug-related reaction, characterized by an acute onset of mainly small non-follicular pustules on an erythematous base. Most cases of acute generalized exanthematous pustulosis (AGEP) clear quickly with a systemic corticosteroid, but severe or recalcitrant cases may need other systemic therapies. In this case, a man in his 40 s with a history of psoriasis consulted a physician about widespread erythema, pustules, target lesions, and fever after the administration of a quadruple antituberculosis drug. Routine laboratory testing revealed elevated white blood cell count and C-reactive protein. The histopathology showed subcorneal pustules, spongiosis as well as lymphocyte and eosinophils infiltration in the dermis. The patient was diagnosed with definitive AGEP according to the diagnostic score from the EuroSCAR study. Cutaneous lesions especially pustules and erythema multiforme-like lesions on the upper arms and palms are crucial for distinguishing AGEP from Generalized pustular psoriasis. The patient was treated with secukinumab as a result of his failure to respond to topical corticosteroids and constrain of systemic steroids. Remission with secukinumab therapy was safe without increased risks of infections. This case indicates that secukinumab is a potential therapy that can rapidly improve the clinical symptoms of AGEP.

The Confusion between Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis as a Cause of Exfoliative Dermatitis: A Case Report

Background: Pustular psoriasis and Acute Generalized Exanthematous Pustulosis (AGEP) are grouped under pustular diseases, in which their clinical manifestations are similar. Those diseases can lead to exfoliative dermatitis. Purpose:To evaluate a specific histopathological examination in differentiating Pustular Psoriasis and AGEP. Case: A 55-year-old woman presented with sudden redness and diffused scaly skin with multiple pustules and also fever. She had taken Cefadroxil 2 days before the scales and pustules appeared. Leukocytosis and histopathological examination results from biopsy supported the diagnosis of AGEP. The patient was then hospitalized and received steroid therapy. Within the first week of tapering off, the scales disappeared but the pustules increased. After such clinical findings, the histopathological examination results were revisited and reassessed. Thus, we considered changing the diagnosis to Pustular Psoriasis, and the therapy was switched to Methotrexate. The patient had a better outcome, and the pustules slowly disappeared entirely. Discussion: It is often difficult to differentiate between the pustules in pustular psoriasis and AGEP unless by thorough history-taking and physical examinations. AGEP is characterized by a widespread of pustules with an acute febrile onset; while pustular psoriasis is an acute variant of psoriasis where pustules are spread over erythematous skin and accompanied by high fever and leukocytosis. Conclusion: Histopathological examination is the gold standard for the establishment of pustular psoriasis diagnosis. The histopathological characteristics of pustular psoriasis and AGEP are difficult to differentiate. Therefore, we need detailed history-taking and physical examination to establish the diagnosis.