Abstract
Hereditary auto-inflammatory syndromes are characterized by aseptic inflammation with recurrent fever and raised acute phase proteins. The skin may be involved in all of these disorders. Recently, many of the responsible genes have been identified. Interleukin-1 turns out to play a central role in their pathogenesis. This knowledge has enabled targeted therapy for most patients. Early recognition by dermatologists can make the difference between a near-normal existence and life-threatening complications.
Translated title of the contribution | Hereditary autoinflammatory syndromes |
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Original language | Dutch |
Pages (from-to) | 523-530 |
Number of pages | 8 |
Journal | Nederlands Tijdschrift voor Dermatologie en Venereologie |
Volume | 22 |
Issue number | 9 |
Publication status | Published - 1 Oct 2012 |
Keywords
- Autoinflammatory diseases
- Genodermatoses
- Inflammasomes
- Periodic fever syndromes