KEGG   DISEASE: 天疱瘡
エントリ  
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  
皮膚および結合組織疾患; 免疫系疾患
階層分類  
ヒト疾患 [BR:jp08402]
 皮膚疾患
  皮膚と軟部組織の疾患
   H01648  天疱瘡
ICD-11 による疾患分類 [BR:jp08403]
 14 Diseases of the skin
  Inflammatory dermatoses
   Immunobullous diseases of the skin
    EB40  Pemphigus
     H01648  天疱瘡
特定疾患 (難病) [jp08407.html]
 H01648
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]
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    
  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
文献    
  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
文献    
  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
文献    
PMID:25405675 (drug)
  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)
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