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American Journal of Clinical Dermatology
Erschienen in: American Journal of Clinical Dermatology 4/2017

01.08.2017 | Review Article

PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment

verfasst von: Massimo Cugno, Alessandro Borghi, Angelo V. Marzano

Erschienen in: American Journal of Clinical Dermatology | Ausgabe 4/2017

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Abstract

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis usually manifesting as skin ulcers with undermined erythematous-violaceous borders. It may be isolated, associated with systemic conditions or occurring in the context of autoinflammatory syndromes such as PAPA (pyogenic arthritis, PG and acne), PASH (PG, acne and suppurative hidradenitis) or PAPASH (pyogenic arthritis, acne, PG and suppurative hidradenitis). From a physiopathological point of view, all these conditions share common mechanisms consisting of over-activation of the innate immune system leading to increased production of the interleukin (IL)-1 family and ‘sterile’ neutrophil-rich cutaneous inflammation. From a genetic point of view, a number of mutations affecting the proteins of the inflammasome complex (the molecular platform responsible for triggering autoinflammation) or the proteins that regulate inflammasome function have been described in these disorders. As these debilitating entities are all associated with the over-expression of IL-1 and tumour necrosis factor (TNF)-α, biological drugs specifically targeting these cytokines are currently the most effective treatments but, given the emerging role of IL-17 in the pathogenesis of these syndromes, IL-17 antagonists may represent the future management of these conditions.
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Metadaten
Titel
PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment
verfasst von
Massimo Cugno
Alessandro Borghi
Angelo V. Marzano
Publikationsdatum
01.08.2017
Verlag
Springer International Publishing
Erschienen in
American Journal of Clinical Dermatology / Ausgabe 4/2017
Print ISSN: 1175-0561
Elektronische ISSN: 1179-1888
DOI
https://doi.org/10.1007/s40257-017-0265-1

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