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Abstract
The emerging group of autoinflammatory diseases (AIDs) is caused by a dysregulation of the innate immune system while lacking the typical footprint of adaptive immunity. A prominent subgroup of AIDs are inflammasomopathies, which are characterised by periodic flares of cutaneous signs as well as systemic organ involvement and fever. The range of possible skin lesions is vast, ranging from urticarial, erysipelas-like and pustular rashes to erythematous patches, violaceous plaques and eventual necrosis and ulceration. This review provides a structured overview of the pathogenesis and the clinical picture with a focus on dermatological aspects of inflammasomopathies. Current treatment options for these conditions are also discussed.
- cryopyrin-associated periodic syndromes
- familial Mediterranean fever
- inflammation
- cytokines
- immune system diseases
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Footnotes
CB and DS are joint first authors.
Handling editor Mary K Crow
Contributors CB and DS contributed equally to this paper and are both corresponding authors. CB, DS and WW wrote the manuscript. MD proofread and edited the manuscript. DS prepared the figures. All authors read and approved the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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