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Cutaneous vasculitis in autoinflammatory diseases.

Ko-Ron Chen1

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Summary
This summary is machine-generated.

Autoinflammatory diseases often involve skin vasculitis. This review highlights cutaneous vasculitis in familial Mediterranean fever, DADA2, and VEXAS syndrome, emphasizing dermatologists' role in early diagnosis.

Keywords:
AIDsDADA2FMFVEXAScutaneous vasculitislivedo racemosa

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Area of Science:

  • Immunology
  • Dermatology
  • Genetics

Background:

  • Autoinflammatory diseases (AIDs) are innate immune system disorders causing recurrent inflammation.
  • Skin lesions and cutaneous vasculitis are common manifestations, sometimes being the most prominent feature.
  • This review focuses on cutaneous vasculitis in three monogenic AIDs: familial Mediterranean fever (FMF), deficiency of adenosine deaminase type 2 (DADA2), and VEXAS syndrome.

Purpose of the Study:

  • To review the association between cutaneous vasculitis and three specific monogenic autoinflammatory diseases.
  • To detail the clinical presentations and diagnostic considerations of cutaneous vasculitis in FMF, DADA2, and VEXAS syndrome.
  • To underscore the importance of dermatological recognition for early diagnosis and management of these conditions.

Main Methods:

  • Literature review focusing on autoinflammatory diseases and associated cutaneous vasculitis.
  • Analysis of clinical and histopathological features of vasculitis in FMF, DADA2, and VEXAS syndrome.
  • Synthesis of diagnostic criteria and management implications for dermatologists.

Main Results:

  • FMF presents with small-vessel vasculitis (similar to IgA vasculitis) or cutaneous arteritis-like nodules.
  • DADA2 exhibits a spectrum of vasculitis, often misdiagnosed as polyarteritis nodosa, with livedo racemosa as a key skin sign.
  • VEXAS syndrome involves diverse skin lesions (Sweet syndrome-like, livedo racemosa), systemic symptoms, and hematologic abnormalities.

Conclusions:

  • Cutaneous vasculitis is a frequent and critical manifestation in FMF, DADA2, and VEXAS syndrome.
  • Early dermatological identification of specific skin lesions can lead to timely diagnosis of these AIDs.
  • Prompt diagnosis and treatment are essential to prevent severe outcomes associated with these potentially fatal diseases.