Vancomycin-induced acute generalized exanthematous pustulosis (AGEP) masquerading septic shock—an unusual presentation of a rare disease

Mawri, Sagger; Jain, Tarun; Shah, Jessica; Hurst, Gina; Swiderek, Jennifer

doi:10.1186/s40560-015-0114-3

Cited by 17 publications

(13 citation statements)

References 11 publications

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“…The first suspicion of a SCAR should warrant a skin biopsy to definitively determine the etiology for this SCAR. Establishing the etiology is an important step for a correct diagnosis since clinical symptoms alone are not reliable for SCAR diagnosis [12][13][14]. Treatment of SCARs begins with discontinuation of the suspected offending drug, but further management should be tailored to the specific reaction with prompt diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Rare and Complicated Overlap of Stevens-Johnson Syndrome and Acute Generalized Exanthematous Pustulosis

Chowdhury

Talib

Patricia

et al. 2021

Cureus

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Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) and acute generalized exanthematous pustulosis (AGEP) are two separate pathological entities of severe cutaneous adverse reactions (SCARs) with different etiologies and treatment strategies. Diagnosis is, however, complicated by the similarity in their clinical presentation. Although there are few claims of AGEP-SJS/TEN overlap, a simultaneous true overlap of SJS/TEN and AGEP has rarely been described in the literature.Here, we report a case study of a 61-year-old female with a known allergy to sulfa drugs presenting with altered mental status, generalized weakness, and erythematous and excoriated purulent wounds. Based on initial workup and extensive consultation, the patient was diagnosed with severe sepsis secondary to diffuse purulent cellulitis, community-acquired pneumonia, and acute renal failure due to prerenal azotemia from dehydration. She was treated with several antibiotics, starting with vancomycin, piperacillin/tazobactam. Six days later, antibiotics were de-escalated to ceftriaxone and metronidazole because of the patient's improved status. The medications were withheld when the patient started developing extensive blistering on day 8. Blood cultures ruled out any bacterial etiology. Skin biopsy confirmed overlapping features of AGEP and SJS/TEN. Due to the uncontrolled progression of her rash, she was transferred to the burn unit of a higher care center. This is potentially the first histologically confirmed case of AGEP-SJS/TEN overlap in the United States. In this case study, a conclusive diagnosis would have never been made without a biopsy, especially because the condition presented clinically as SJS/TEN. We, therefore, recommend considering a potential overlap of multiple pathologies at each presentation or suspicion of a SCAR and performing an early skin biopsy in order to provide definitive diagnosis and treatment.

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Section: Discussionmentioning

confidence: 99%

Rare and Complicated Overlap of Stevens-Johnson Syndrome and Acute Generalized Exanthematous Pustulosis

Chowdhury

Talib

Patricia

et al. 2021

Cureus

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“…5 However, only 15 adult cases of AGEP with haemodynamic instability have been reported in the literature. [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] The pathophysiology of AGEP is a drug-specific CD4+ and CD8+ T cell response that results in increased production of IL-8, a potent neutrophilattracting chemokine. 25 Other inflammatory cytokines, including IL-17 and IL-22, also have a role in the pathophysiology of AGEP, and may be involved in the systemic complications of AGEP.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and management of acute generalized exanthematous pustulosis with haemodynamic instability

O'Brian

Carr

Thomas

et al. 2021

Skin Health and Disease

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Background: Acute generalized exanthematous pustulosis (AGEP) is a severe pustular drug eruption with rare reports of haemodynamic instability. Objective: To describe the clinical characteristics, management, and outcomes of patients with AGEP-associated haemodynamic instability. Methods: This retrospective case series identified adult patients diagnosed with AGEP who had haemodynamic instability from November 2012 to February 2020 that were seen at two academic teaching hospitals with roles as a burn centre and tertiary referral centre at the University of Texas Southwestern Medical Center in Dallas, TX USA. Patients with a discharge diagnosis of AGEP that had haemodynamic instability during their eruption were included. Patients with a history of psoriasis, presentations thought to be a flare of generalized pustular psoriasis, or concurrent infection during eruption were excluded. AGEP with haemodynamic instability was characterized by degree of hypotension, dermatologic phenotype at time of dermatologic consultation, and management approach. Results: This study included 19 patients with AGEP-associated haemodynamic instability (mean age, 52 years; age range, 29-76 years; 11 (58%) female). Patients were classified on a spectrum of haemodynamic instability; three had sustained hypotension, 10 had hypotension with organ dysfunction, and six had shock. Patients with AGEP-associated haemodynamic instability had a range of dermatologic phenotypes at initial consultation: subtle exanthematous eruption with minimal pustules, typical eruption with pustules and flexural predominance, and severe eruption with features of Stevens-Johnson syndrome. Both topical and systemic corticosteroids were used for treatment of several patients. Of the patients that required vasopressors and received systemic steroids, the majority were off vasopressors within 24 h of steroid initiation. Conclusion: Approximately 22% of patients presenting with AGEP to a tertiary referral center had haemodynamic instability. Clinicians should beThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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“…However, to the best of our knowledge, there have been only a few reported cases of AGEP with such severe presentation and none of these cases were associated with the use of erythromycin and fluconazole. 7 8 In most of the severe presentations, vancomycin was the culprit medication. 7 8 It is possible that the combined effect of erythromycin and fluconazole which are both hepatically metabolised and alter each other's metabolism leading to increased drug levels resulted in the drug reaction being severe with systemic involvement as an affect.…”

Section: Discussionmentioning

confidence: 99%

An illusion of septic shock: acute generalised exanthematous pustulosis with multiorgan dysfunction

2017

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Acute generalised exanthematous pustulosis (AGEP) is a rare cutaneous eruption, most often caused by commonly used antibiotics. It is characterised by an acute onset of non-follicular sterile pustular rash and erythema within hours or days of drug exposure and usually resolves spontaneously within 1–2 weeks once the drug is discontinued. Haemodynamic involvement in the form of shock is rare. Here, we present a severe case of AGEP, manifesting with systemic involvement and haemodynamic instability resulting in shock with multiorgan dysfunction. The associated drugs were erythromycin and fluconazole with a possible combined effect of these two drugs that resulted in systemic involvement. Our patient improved markedly, both haemodynamically and dermatologically, after discontinuation of the drugs and with systemic steroid therapy.

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Vancomycin-induced acute generalized exanthematous pustulosis (AGEP) masquerading septic shock—an unusual presentation of a rare disease

Cited by 17 publications

References 11 publications

Rare and Complicated Overlap of Stevens-Johnson Syndrome and Acute Generalized Exanthematous Pustulosis

Rare and Complicated Overlap of Stevens-Johnson Syndrome and Acute Generalized Exanthematous Pustulosis

Clinical characteristics and management of acute generalized exanthematous pustulosis with haemodynamic instability

An illusion of septic shock: acute generalised exanthematous pustulosis with multiorgan dysfunction

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