Autoinflammatory diseases accompanied by vasculitis
10.3760/cma.j.issn.0412-4030.2019.05.013
- VernacularTitle:伴有血管炎的自身炎症性疾病
- Author:
Wei LIU
1
;
Jiawei LIU
;
Yuetong QIAN
;
Donglai MA
Author Information
1. 中国医学科学院北京协和医学院北京协和医院皮肤科 100730
- Keywords:
Hereditary autoinflammatory diseases;
Vasculitis
- From:
Chinese Journal of Dermatology
2019;52(5):340-342
- CountryChina
- Language:Chinese
-
Abstract:
Autoinflammatory diseases are a group of inherited disorders characterized by dysregulation of the immune system,periodic fever and reccurrence of inflammation.The common rashes of autoinflammatory diseases include acne,pyoderma gangrenosum,erysipelas,urticaria,ichthyosis,pustulosis,while vasculitis is rare.This review summarizes autoinflammatory diseases accompanied by vasculitis.Familial Mediterranean fever mainly manifests as small-and medium-vessel vasculitis such as Henoch-Sch(o)nlein purpura and polyarteritis nodosa,while large-vessel vasculitis is common in Blau syndrome.Small-and medium-artery vasculitis can occur in patients with deficiency of adenosine deaminase 2 as well,so it should be differentiated from polyarteritis nodosa in clinical practice.Stimulator of interferon gene-associated vasculopathy with onset in infancy mainly presents as cutaneous small-vessel vasculitis and vasculopathy.
