Acute Generalized Exanthematous Pustulosis: Pathogenesis, Genetic Background, Clinical Variants and Therapy.

Feldmeyer, Laurence; Heidemeyer, Kristine; Yawalkar, Nikhil (2016). Acute Generalized Exanthematous Pustulosis: Pathogenesis, Genetic Background, Clinical Variants and Therapy. International journal of molecular sciences, 17(8) Molecular Diversity Preservation International MDPI 10.3390/ijms17081214

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Acute generalized exanthematous pustulosis (AGEP) is a severe, usually drug-related reaction, characterized by an acute onset of mainly small non-follicular pustules on an erythematous base and spontaneous resolution usually within two weeks. Systemic involvement occurs in about 20% of cases. The course is mostly benign, and only in rare cases complications lead to life-threatening situations. Recent studies highlight the importance of genetic variations in interleukin-36 receptor antagonist gene (IL-36RN) in the pathogenesis of this disease. The physiopathology of AGEP remains unclear, but an involvement of innate and acquired immune cells together with resident cells (keratinocytes), which recruit and activate neutrophils via production of cytokines/chemokines such as IL-17, IL-36, granulocyte-macrophage colony-stimulating factor (GM-CSF), tumor necrosis factor alpha (TNFα) and chemokine (C-X-C motif) ligand 8 (CXCL8)/IL-8, has been postulated. Treatment is based on the removal of the causative drug, supportive care, infection prevention and use of potent topical or systemic steroids.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology

UniBE Contributor:

Feldmeyer, Laurence; Heidemeyer, Kristine and Yawalkar, Nikhil

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1661-6596

Publisher:

Molecular Diversity Preservation International MDPI

Language:

English

Submitter:

Sandra Nyffenegger

Date Deposited:

28 Feb 2017 09:55

Last Modified:

04 Oct 2017 15:34

Publisher DOI:

10.3390/ijms17081214

PubMed ID:

27472323

Uncontrolled Keywords:

acute generalized exanthematous pustulosis; dermatology; drug reaction; skin

BORIS DOI:

10.7892/boris.93458

URI:

https://boris.unibe.ch/id/eprint/93458

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