KEGG   DISEASE: 類天疱瘡
エントリ  
H01650                                                             

名称    
類天疱瘡
概要    
Pemphigoid diseases are group of autoimmune blistering disorders of the skin and mucous membranes characterized by autoantibodies directed against structural proteins of the dermal-epidermal junction that clinically can manifest with urticarial lesions, tense blisters, and erosions which may involve the mucous membranes. Diseases in this group include bullous pemphigoid (BP), dermatitis herpetiformis, mucous membrane pemphigois, linear IgA bullous dermatosis, herpes gestationis, and epidermolysis bullosa acquisita. BP is the most common acquired bullous autoimmune dermatosis disease. It is primarily a disease of the elderly with an equal incidence in men and women. The clinical characteristics are the formation of tense blisters and pruritic urticarial erythema. Histopathologically, subepidermal blister formation associated with eosinophil infiltration is commonly observed in BP. The main autoantigens targeted by BP autoantibodies are hemidesmosomal transmembrane collagen XVII (COL17, also known as BP180/BPAG2) and the intracytoplasmic plakin family protein BP230. Most of these antibodies belong to the immunoglobulin G class. Diagnosis of BP relies on the clinical and histological aspects, but the most reliable clues are given by direct and indirect immunofluorescence. Three types of drugs, with distinct mechanisms of action, are used to treat BP: anti-inflammatory drugs, drugs designed to reduce the production of pathogenic antibodies, and treatments that increase the elimination of pathogenic antibodies from the serum of patients, such as high-dose intravenous immunoglobulin (IVIG) therapy and therapeutic plasma exchange.
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
   H01650  類天疱瘡
ICD-11 による疾患分類 [BR:jp08403]
 14 Diseases of the skin
  Inflammatory dermatoses
   Immunobullous diseases of the skin
    EB41  Pemphigoid
     H01650  類天疱瘡
特定疾患 (難病) [jp08407.html]
 H01650
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:
BP180 [HSA:1308] [KO:K07603]
BP230 [HSA:667] [KO:K10382]
LAMA3 [HSA:3909] [KO:K06240]
LAMB3 [HSA:3914] [KO:K06244]
ITGA6 [HSA:3655] [KO:K06485]
ITGB4 [HSA:3691] [KO:K06525]
COL7A1 [HSA:1294] [KO:K16628]
TGM3 [HSA:7053] [KO:K05620]
リンク   
ICD-11: EB41
ICD-10: L12
MeSH: D010391
文献    
  著者
Ujiie H
  タイトル
IgE autoantibodies in bullous pemphigoid: supporting role, or leading player?
  雑誌
J Dermatol Sci 78:5-10 (2015)
DOI:10.1016/j.jdermsci.2015.03.002
文献    
  著者
Fuertes de Vega I, Iranzo-Fernandez P, Mascaro-Galy JM
  タイトル
Bullous pemphigoid: clinical practice guidelines.
  雑誌
Actas Dermosifiliogr 105:328-46 (2014)
DOI:10.1016/j.ad.2012.10.022
文献    
  著者
Danescu S, Chiorean R, Macovei V, Sitaru C, Baican A
  タイトル
Role of physical factors in the pathogenesis of bullous pemphigoid: Case report series and a comprehensive review of the published work.
  雑誌
J Dermatol 43:134-40 (2016)
DOI:10.1111/1346-8138.13031
文献    
PMID:24434358 (description, autoantigen)
  著者
Baum S, Sakka N, Artsi O, Trau H, Barzilai A
  タイトル
Diagnosis and classification of autoimmune blistering diseases.
  雑誌
Autoimmun Rev 13:482-9 (2014)
DOI:10.1016/j.autrev.2014.01.047
LinkDB    

» English version

DBGET integrated database retrieval system