アブストラクト
Japanese
| Title | 蕁麻疹様血管炎とその類縁疾患 |
|---|---|
| Subtitle | 総説 |
| Authors | 池田高治 |
| Authors (kana) | |
| Organization | 東北医科薬科大学医学部皮膚科学教室 |
| Journal | 日本小児アレルギー学会誌 |
| Volume | 36 |
| Number | 3 |
| Page | 248-256 |
| Year/Month | 2022 / |
| Article | 報告 |
| Publisher | 日本小児アレルギー学会 |
| Abstract | 「抄録」 : 蕁麻疹様血管炎は, 蕁麻疹よりも長時間持続し, 掻痒や灼熱感を伴い, 紫斑を残して消退する蕁麻疹様紅斑を呈する. 皮膚真皮浅層に分布する小血管特に細静脈 (postcapillary venule) に白血球破砕性血管炎を示す血管炎の一型である. 血清補体価の低下を伴う低補体血症性蕁麻疹様血管炎と血清補体価が基準範囲の正補体血症性蕁麻疹様血管炎に分類される. 低補体血症性蕁麻疹様血管炎の発症機序として, 抗C1q抗体による免疫複合体形成での補体活性化が想定されている. 皮膚血管炎を示す小児症例の多くはIgA血管炎であり, 小児発症の蕁麻疹様血管炎は稀とされているが, 一定の割合で蕁麻疹様血管炎の症例が診断されている. その中には, DNASE1L3 mutationが同定され, 全身性エリテマトーデスを発症する症例もある. 蕁麻疹様紅斑以外にも, 予後を左右する慢性閉塞性肺疾患や種々の型の糸球体腎炎, 上強膜炎や結膜炎など, 皮膚外症状を伴うことがある. 蕁麻疹・全身性エリテマトーデス・自己炎症性疾患などとの鑑別を行う必要がある. |
| Practice | 臨床医学：内科系 |
| Keywords | 自己炎症性疾患, 低補体血症性蕁麻疹様血管炎, DNASE1L3 機能喪失変異, 蕁麻疹, 蕁麻疹様血管炎, autoinflammatory diseases, hypocomplementemic urticarial vasculitis, loss-of-function mutation of the DNASE1L3 gene, urticaria, urticarial vasculitis |
English
| Title | Urticarial vasculitis and related diseases |
|---|---|
| Subtitle | |
| Authors | Takaharu Ikeda |
| Authors (kana) | |
| Organization | Division of Dermatology, Tohoku Medical and Pharmaceutical University |
| Journal | The Japanese Journal of Pediatric Allergy and Clinical Immunology |
| Volume | 36 |
| Number | 3 |
| Page | 248-256 |
| Year/Month | 2022 / |
| Article | Report |
| Publisher | Japanese Society of Pediatric Allergy and Clinical Immunology |
| Abstract | [Summary] : Urticarial vasculitis presents urticaria-like eruptions that typically last longer than urticaria with pruritus and burning sensation and resolve with residual purpura or hyperpigmentation. It histologically shows leukocytoclastic vasculitis that affects small vessels in the upper dermis, especially postcapillary venules. It is classified into hypocomplementemic urticarial vasculitis with a decrease in serum complement titer and normocomplementemic urticarial vasculitis with a reference range of serum complement titer. Complement activation by immune complex formation due to anti-C1q antibodies is assumed to be a pathogenic mechanism of hypocomplementemic urticarial vasculitis syndrome. Most pediatric cases with cutaneous vasculitis are diagnosed with IgA vasculitis, and childhood-onset urticarial vasculitis is thought to be rare. However, there are cases of urticarial vasculitis with a certain probability. In some childhood-onset urticarial vasculitis cases, it was reported that mutations in DNASE1L3 were identified and they concurrent systemic lupus erythematosus. Urticarial vasculitis may present extracutaneous symptoms such as chronic obstructive pulmonary disease that affects their prognosis, various types of glomerulonephritis, episcleritis, and conjunctivitis. It is necessary to distinguish it from urticaria, systemic lupus erythematosus, autoinflammatory diseases, and so on. |
| Practice | Clinical internal medicine |
| Keywords | autoinflammatory diseases, hypocomplementemic urticarial vasculitis, loss-of-function mutation of the DNASE1L3 gene, urticaria, urticarial vasculitis |
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残りの49件を表示する
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