Acute Generalized Exanthematous Pustulosis: Clinical Characteristics, Pathogenesis, and Management.

Acute generalized exanthematous pustulosis Cutaneous adverse drug reactions Innate cytokines Innate immunity

Journal

Dermatology (Basel, Switzerland)
ISSN: 1421-9832
Titre abrégé: Dermatology
Pays: Switzerland
ID NLM: 9203244

Informations de publication

Date de publication:
2023
Historique:
received: 25 10 2022
accepted: 13 01 2023
medline: 7 6 2023
pubmed: 27 1 2023
entrez: 26 1 2023
Statut: ppublish

Résumé

Acute generalized exanthematous pustulosis (AGEP) is a potentially severe adverse cutaneous drug reaction, which typically occurs within 24-48 h after the intake of the culprit drug. AGEP is characterized by numerous sterile subcorneal pustules on erythematous skin and in less than a third of cases it can be associated with organ manifestations possibly leading to life-threatening symptoms (e.g., cholestasis, nephritis, and lung and bone marrow involvement). In contrast to generalized pustular psoriasis, it can involve mucosal regions and typically resolves rapidly if the culprit drug is removed, and adequate therapy with topical or systemic steroids administered. Diagnosis based on patient history, clinical signs, and characteristic cutaneous histology is rarely challenging. Identification of the culprit drug may be aided by patch testing or lymphocyte transformation tests that are of limited value. Recent experimental data reviewed herein are supportive of an early role of drug-induced innate immune activation and innate cytokines such as interleukin (IL)-1, IL-36, and IL-17 in the pathogenesis of AGEP. This explains the rapid onset and neutrophilic character of the cutaneous inflammation, but also provides new avenues for in vitro tests aimed at better identifying the culprit drug.

Sections du résumé

BACKGROUND BACKGROUND
Acute generalized exanthematous pustulosis (AGEP) is a potentially severe adverse cutaneous drug reaction, which typically occurs within 24-48 h after the intake of the culprit drug.
SUMMARY CONCLUSIONS
AGEP is characterized by numerous sterile subcorneal pustules on erythematous skin and in less than a third of cases it can be associated with organ manifestations possibly leading to life-threatening symptoms (e.g., cholestasis, nephritis, and lung and bone marrow involvement). In contrast to generalized pustular psoriasis, it can involve mucosal regions and typically resolves rapidly if the culprit drug is removed, and adequate therapy with topical or systemic steroids administered. Diagnosis based on patient history, clinical signs, and characteristic cutaneous histology is rarely challenging. Identification of the culprit drug may be aided by patch testing or lymphocyte transformation tests that are of limited value.
KEY MESSAGES CONCLUSIONS
Recent experimental data reviewed herein are supportive of an early role of drug-induced innate immune activation and innate cytokines such as interleukin (IL)-1, IL-36, and IL-17 in the pathogenesis of AGEP. This explains the rapid onset and neutrophilic character of the cutaneous inflammation, but also provides new avenues for in vitro tests aimed at better identifying the culprit drug.

Identifiants

pubmed: 36702114
pii: 000529218
doi: 10.1159/000529218
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

328-333

Informations de copyright

© 2023 S. Karger AG, Basel.

Auteurs

Pia-Charlotte Stadler (PC)

Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, Germany.

Anna Oschmann (A)

Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, Germany.

Katrin Kerl-French (K)

Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, Germany.

Julia-Tatjana Maul (JT)

Department of Dermatology, University of Zurich, Zurich, Switzerland.
Faculty of Medicine, University of Zurich, Zurich, Switzerland.

Eva Maria Oppel (EM)

Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, Germany.

Barbara Meier-Schiesser (B)

Department of Dermatology, University of Zurich, Zurich, Switzerland.
Faculty of Medicine, University of Zurich, Zurich, Switzerland.

Lars Einar French (LE)

Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, Germany.
Department of Dermatology and Cutaneous Surgery, University of Miami, Miller School of Medicine, Miami, Florida, USA.

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Classifications MeSH