| H01648 | |
| H番号 | H01648 |
| 名称 | 天疱瘡 |
| 概要 | Pemphigus is an autoimmune blistering disease of the skin and mucous membranes characterized by circulating autoantibodies that target desmosomal components as well as other epithelial antigens and impair keratinocytes adhesion with subsequent acantholysis. Although the precise pathomechanisms of pemphigus have not yet been fully elucidated, recent studies have shown that desmosome disassembling involves not only the direct inhibition of desmosome formation caused by IgGs, but also desmosome remodeling. Pemphigus can be divided into non-inflammatory types, which are the major and common types, and inflammatory types, which are much less common and include necrolytic (apoptotic) type. Non-inflammatory pemphigus includes two types: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The former is caused by autoantibodies to desmoglein (Dsg) 3 only or to both Dsg1 and Dsg3 and generates clinically multiple flaccid blisters and erosions due to acantholysis at the lower, typically along the suprabasal, layers in the epidermis and mucous membranes. The latter is caused by autoantibodies to Dsg1 and clinically characterized by very fragile superficial blisters and large leafy scales due to acantholysis at the granular cell layer in the epidermis, but not observed in the mucous membranes. Inflammatory types of pemphigus include pemphigus herpetiformis (PH), pemphigus vegetans (PVeg) and the so-called paraneoplastic pemphigus (PP). Because pemphigus is an autoimmune disease, the most common treatment is the administration of oral steroids to inhibit antibody production, combined with the use of topical agents to prevent infection, protect the eroded areas and promote epithelialization. Other concomitant therapy can include the use of immunosuppressants, plasma exchange, and the administration of high-dose intravenous immunoglobulin (IVIG). |
| カテゴリ | 免疫系疾患; 皮膚疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | デキサメタゾン [DR:D00292] デキサメタゾンリン酸エステルナトリウム [DR:D00975] ベタメタゾンリン酸エステルナトリウム [DR:D00972] トリアムシノロンアセトニド [DR:D00983] モメタゾンフランカルボン酸エステル [DR:D00690] ヒドロコルチゾン [DR:D00088] ヒドロコルチゾンコハク酸エステルナトリウム [DR:D00978] コルチゾン酢酸エステル [DR:D00973] トリアムシノロン [DR:D00385] ベタメタゾン [DR:D00244] プレドニゾロン [DR:D00472] プレドニゾロンコハク酸エステルナトリウム [DR:D01239] メチルプレドニゾロン [DR:D00407] メチルプレドニゾロン酢酸エステル [DR:D00979] デキサメタゾンプロピオン酸エステル [DR:D01632] ベタメタゾン吉草酸エステル [DR:D01357] ベタメタゾンジプロピオン酸エステル [DR:D01637] ベタメタゾン酪酸エステルプロピオン酸エステル [DR:D02032] ジフロラゾン酢酸エステル [DR:D01327] クロベタゾールプロピオン酸エステル [DR:D01272] ジアフェニルスルホン [DR:D00592] リツキシマブ [DR:D02994] ポリエチレングリコール処理人免疫グロブリン [DR:D08783] 乾燥ポリエチレングリコール処理人免疫グロブリン [DR:D08788] |
| コメント | Autoantigen: DSG1 [HSA:1828] [KO:K07596] DSG3 [HSA:1830] [KO:K07598] |
| リンク | ICD-11: EB40 ICD-10: L10 MeSH: D010392 |
| 文献 | PMID:25078507 著者 Kitajima Y タイトル 150(th) anniversary series: Desmosomes and autoimmune disease, perspective of dynamic desmosome remodeling and its impairments in pemphigus. 雑誌 Cell Commun Adhes 21:269-80 (2014) DOI:10.3109/15419061.2014.943397 PMID:24909210 著者 Amagai M, Tanikawa A, Shimizu T, Hashimoto T, Ikeda S, Kurosawa M, Niizeki H, Aoyama Y, Iwatsuki K, Kitajima Y タイトル Japanese guidelines for the management of pemphigus. 雑誌 J Dermatol 41:471-86 (2014) DOI:10.1111/1346-8138.12486 PMID:27329525 著者 Celentano A, Cirillo N タイトル Desmosomes in disease: a guide for clinicians. 雑誌 Oral Dis 23:157-167 (2017) DOI:10.1111/odi.12527 PMID:25405675 著者 Hooten J, Hall R 3rd, Cardones A タイトル Updates on the management of autoimmune blistering diseases. 雑誌 Skin Therapy Lett 19:1-6 (2014) |