Acute generalised exanthematous pustulosis associated with shock

Abstract

A 23-year-old man with a history of end-stage renal disease was admitted to the hospital due to fever and shock, which occurred during his dialysis. One week prior, he developed an erythematous rash on his chest, face and back, associated with generalised eruption of pustules. In hospital, his status did not improve with norepinephrine and empirical broad-spectrum antibiotics. Following this, methylprednisolone was administered with remarkable improvement. Cultures revealed no infectious aetiology. Based on the morphology of the rash and a compatible skin biopsy, the diagnosis of acute generalised exanthematous pustulosis (AGEP) was established and considered the cause of his shock. The causative agent of his AGEP remained unknown. AGEP is a rare condition, most frequently associated with drug exposure. The removal of the offending agent is the treatment of choice. It can be complicated by shock in rare cases. In that scenario, systemic corticosteroids seem to improve outcomes greatly.

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

“…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.…”

“…Systemic involvement of AGEP has been described, most commonly involving the liver and kidneys and less often the lungs 5 . However, only 15 adult cases of AGEP with haemodynamic instability have been reported in the literature 9–23 …”

“…[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] These reports document cases of AGEP with hypotension requiring intravenous fluid resuscitation and some necessitating vasopressor support. [9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] Since AGEP with haemodynamic instability is rarely encountered, there is a lack of information regarding dermatologic presentation, laboratory findings, and treatment of these patients. Furthermore, two of the largest retrospective studies documenting systemic involvement of AGEP did not collect data related to blood pressure or hypotension.…”

“…Although systemic involvement of AGEP is uncommon, there are rare reports of AGEP‐associated haemodynamic instability in the literature 9–23 . These reports document cases of AGEP with hypotension requiring intravenous fluid resuscitation and some necessitating vasopressor support 9–23 .…”

“…Although systemic involvement of AGEP is uncommon, there are rare reports of AGEP‐associated haemodynamic instability in the literature 9–23 . These reports document cases of AGEP with hypotension requiring intravenous fluid resuscitation and some necessitating vasopressor support 9–23 . Since AGEP with haemodynamic instability is rarely encountered, there is a lack of information regarding dermatologic presentation, laboratory findings, and treatment of these patients.…”