scholarly journals Clinicopathologic Manifestations of 36 Korean Patients with Acute Generalized Exanthematous Pustulosis: A Case Series and Review of the Literature

2010 ◽  
Vol 22 (2) ◽  
pp. 163 ◽  
Author(s):  
Min Jee Choi ◽  
Hei Sung Kim ◽  
Hyun Jeong Park ◽  
Chul Jong Park ◽  
Jeong Deuk Lee ◽  
...  
2019 ◽  
Vol 7 ◽  
pp. 2050313X1982643
Author(s):  
Julia N Mayba ◽  
C Nicole Hawkins

We present a case of Sneddon-Wilkinson disease in a 52-year-old female at her first presentation to dermatology. Outlined in the case are various investigations undertaken at this initial presentation, including rheumatologic and hematologic malignancy markers, which identified immunoglobulin A gammopathy. The systemic and topical therapies used to treat the patient’s condition are described, as well as her response to these treatments. In this discussion, we explain the epidemiology, pathophysiology, and clinical presentation of Sneddon-Wilkinson disease. Various medical conditions having known association with Sneddon-Wilkinson disease are discussed, including immunoglobulin A or immunoglobulin G monoclonal gammopathies and lymphoproliferative disorders. A comprehensive differential diagnosis for Sneddon-Wilkinson disease is provided, including immunoglobulin A pemphigus, acute generalized exanthematous pustulosis and pustular psoriasis, among others. We describe the systemic and topical therapy options for the treatment of Sneddon-Wilkinson disease, of which first line treatment is systemic dapsone. This patient serves as an excellent case of Sneddon-Wilkinson disease with unexpected immunoglobulin A gammopathy.


2008 ◽  
Vol 30 (5) ◽  
pp. 930-940 ◽  
Author(s):  
Andrea Paradisi ◽  
Leonardo Bugatti ◽  
Teresa Sisto ◽  
Giorgio Filosa ◽  
Pier Luigi Amerio ◽  
...  

2022 ◽  
Vol 11 (2) ◽  
pp. 397
Author(s):  
Enriqueta Vallejo-Yagüe ◽  
Adrian Martinez-De la Torre ◽  
Omar S. Mohamad ◽  
Shweta Sabu ◽  
Andrea M. Burden

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.


2013 ◽  
Vol 17 (6) ◽  
pp. 414-418 ◽  
Author(s):  
Kristy Bailey ◽  
Daniel Mckee ◽  
Judy Wismer ◽  
Neil Shear

Background: Acute generalized exanthematous pustulosis (AGEP) is a rare drug eruption presenting with an acute, extensive formation of nonfollicular sterile pustules on an erythematous and edematous base. Typically, the rash is accompanied by fever and leukocytosis, with spontaneous resolution in < 15 days. The incidence of AGEP is estimated at one to five cases per million people per year. Only 18% of these are from nonantibiotics. Hydroxychloroquine (HCQ) is an antimalarial agent that is also used to treat various dermatologic and rheumatologic conditions. Objective: We report the first observation in Canada of a patient with AGEP induced by HCQ. Methods and Results: AGEP was diagnosed in a 48-year-old female who had been taking HCQ for 2 weeks and then developed a diffuse erythematous and edematous pustular eruption. Clinical and pathologic findings were consistent with a diagnosis of AGEP. The patient was treated with steroids and supportive measures. The rash resolved after 18 days and a complicated course in hospital. Conclusion: AGEP is a rare drug eruption, usually to antibiotics. We report the first case in Canada of AGEP as an adverse reaction to HCQ. Clinicians should keep in mind the possibility of this severe skin eruption.


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