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The systemic autoinflammatory disorders for dermatologists. Part 1: overview
Author(s) -
Oldham J.,
Lachmann H. J.
Publication year - 2020
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/ced.14250
Subject(s) - medicine , intensive care medicine , amyloidosis , dermatology , pathology
Summary The systemic autoinflammatory disorders (SAIDs) or periodic fever syndromes are disorders of innate immunity, which can be inherited or acquired. They are almost all very rare and easily overlooked; typically, patients will have seen multiple specialities prior to diagnosis, so a high level of clinical suspicion is key. It is important to note that these are ‘high‐value’ diagnoses as the majority of these syndromes can be very effectively controlled, dramatically improving quality of life and providing protection against the development of irreversible complications such as AA amyloidosis. In this article, we take an overview of SAIDs and look at the common features; in Part 2, we take a more in‐depth look at the better recognized or more dermatologically relevant conditions.