Acute generalized exanthematous pustulosis: an overview of the clinical, immunological and diagnostic concepts

Abstract

Acute generalized exanthematous pustulosis: an overview of the clinical, immunological and diagnostic concepts Acute generalized exanthematous pustulosis (AGEP) is a significant adverse cutaneous reaction, most often provoked by drugs and acute infections. The recognition of AGEP is important, in order to avoid confusion with a systemic infection and consequently to avoid incorrect treatment. The clinical hallmark is the presence of multiple disseminated sterile pustules on an erythematous background, associated with fever and a massive neutrophilia and sometimes eosinophilia. The disease is characterised by an acute onset and a spontaneous resolution within 2 weeks. The involvement of drug-specific T cells in the pathomechanism can be confirmed by positive skin patch tests and lymphocyte transformation tests. In this review, we highlight the main clinical, pathophysiological and diagnostic aspects of this peculiar form of drug allergy.

Baboon syndrome: an unusual complication arising from antibiotic treatment of tonsillitis and review of the literature

“…The face is frequently involved 23. Skin symptoms usually arise within hours,24 the patient is feverish and the neutrophil count is elevated 23. Resolution is typically associated with desquamation 23.…”

“…Skin symptoms usually arise within hours,24 the patient is feverish and the neutrophil count is elevated 23. Resolution is typically associated with desquamation 23. AGEP has similar histological features to baboon syndrome/SDRIFE, with subcorneal pustules, papillary oedema and perivascular infiltrates of neutrophils and eosinophils 23…”

Acute generalized exanthematous pustulosis after ceftriaxone use resembling sepsis

“…Acute generalized exanthematous pustulosis is a cutaneous adverse reaction that can be provoked by drugs and acute infections [8]. To the best of our knowledge, there is an only one case report of recurrent urinary tract infections caused by E. coli that caused AGEP [9].…”

“…Corticosteroids (oral, topical, systemic) may be given, but no significant difference has been reported between treatment regimens in the course and recovery period [11]. In general, specific therapy is not necessary [8]. The patient had a history of drug-related rash 6 months previously but she did not recall the details of this event.…”

T cell–mediated acute localized exanthematous pustulosis caused by finasteride

“…Allergic workup in patients with AGEP might show positive skin test reactions of the delayed type with positive patch test results, reactivity in lymphocytic stimulation assays, or both, as seen in this case. 6 The clinical differential diagnosis includes follicular eruptions, such as bacterial folliculitis, furunculosis, acne and acneiform pustules, localized pustular contact dermatitis, dermatophyte infections, pyoderma vegetans, varicella, Kaposi varicelliform eruption, Sweet syndrome, bullous impetigo, impetiginized eczema, pustular erythema multiforme, pemphigus foliaceus and other autoimmune bullous disorders, infantile chronic acropustulosis, migratory necrolytic eruption of glucagonoma, bowel bypass syndrome, Behcet disease, or staphylococcal scalded skin syndrome and others. Most of them can easily be differentiated from AGEP based on clinical appearance, clinical course, and histology, but some diseases remain in which differentiation from AGEP can cause problems.…”