エントリ |
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名称 |
皮膚エリテマトーデス; 紅斑性狼瘡 |
概要 |
Cutaneous lupus erythematosus (CLE) is the skin-related form of lupus erythematosus (LE), with a broad spectrum of clinical manifestations and a variable course. CLE is a frequent finding in patients with systemic lupus erythematosus (SLE) and can also exist as a single entity without associated systemic autoimmunity (LE-specific skin disease). Despite ongoing research into the cause of CLE, it remains unclear how CLE relates to SLE pathogenesis. LE-specific skin disease includes the subtypes of CLE such as acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE), and intermittent CLE (ICLE). ACLE is characterized by malar rash or maculopapular eruption on sun exposed areas. SCLE characteristically presents as annular or psoriasiform plaques in a photo distribution. CCLE can be further divided into discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain LE (CLE), and LE tumidus (LET). The pathogenesis of CLE is multifactorial and involves genetic predisposition, environmental triggers, and abnormalities in the immune response. It has been reported that many genetic risk factors for CLE involve HLA or interferon-related pathways. Corticosteroids and antimalarials were considered as the most suitable and effective systemic drugs for CLE patients.
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カテゴリ |
免疫系疾患; 皮膚疾患
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階層分類 |
ヒト疾患 [BR:jp08402]
免疫系疾患
アレルギーと自己免疫疾患
H01595 皮膚エリテマトーデス
皮膚疾患
皮膚と軟部組織の疾患
H01595 皮膚エリテマトーデス
ICD-11 による疾患分類 [BR:jp08403]
14 Diseases of the skin
Inflammatory dermatoses
Cutaneous lupus erythematosus
EB50 Subacute cutaneous lupus erythematosus
H01595 皮膚エリテマトーデス
EB51 Chronic cutaneous lupus erythematosus
H01595 皮膚エリテマトーデス
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治療薬 |
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コメント |
See also H00080 Systemic lupus erythematosus, and H00291 Familial chilblain lupus (FCL).
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リンク |
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文献 |
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著者 |
Stannard JN, Kahlenberg JM |
タイトル |
Cutaneous lupus erythematosus: updates on pathogenesis and associations with systemic lupus. |
雑誌 |
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文献 |
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著者 |
Kuhn A, Sticherling M, Bonsmann G |
タイトル |
Clinical manifestations of cutaneous lupus erythematosus. |
雑誌 |
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文献 |
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著者 |
Chasset F, Arnaud L, Costedoat-Chalumeau N, Zahr N, Bessis D, Frances C |
タイトル |
The effect of increasing the dose of hydroxychloroquine (HCQ) in patients with refractory cutaneous lupus erythematosus (CLE): An open-label prospective pilot study. |
雑誌 |
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LinkDB |
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