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

Abstract

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.

Sudden Conjunctivitis, Lymphopenia, and Rash Combined With Hemodynamic Changes (SCoRCH) After Trimethoprim-Sulfamethoxazole Use

“…Notably, the sudden onset of symptoms within hours of oral rechallenge was a unique feature of this reaction that is not common in other drug reactions apart from AGEP and type 1 HSRs . Although subtle presentations of AGEP with generalized erythema, minimal pustules, and hemodynamic changes have been described, results of skin biopsies demonstrate typical features of AGEP . Although only 1 patient in the cohort underwent a skin biopsy, the nonspecific findings on histopathologic evaluation may have helped to exclude other drug reactions.…”

“…13 Although subtle presentations of AGEP with generalized erythema, minimal pustules, and hemodynamic changes have been described, results of skin biopsies demonstrate typical features of AGEP. 14,15 Although only 1 patient in the cohort underwent a skin biopsy, the nonspecific findings on histopathologic evaluation may have helped to exclude other drug reactions. Lastly, the differential diagnosis included eruptions seen in viral infections, toxin-mediated syndromes, Kawasaki disease, and COVID-19 multiorgan inflammatory syndrome.…”

Acute generalized exanthematous pustulosis: Clinico‐biological profile and inducing drugs in 83 patients

“…4 Hemodynamic instability may occur, probably due to a skin inflammation-related capillary leak syndrome. 9 EBV replication, not previously explored in AGEP, was frequent, even in AGEP cases without overlap with DRESS.…”