類天疱瘡

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.

皮膚および結合組織疾患; 免疫系疾患

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]

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]

PMID:25797172

Ujiie H

IgE autoantibodies in bullous pemphigoid: supporting role, or leading player?

J Dermatol Sci 78:5-10 (2015)
DOI:10.1016/j.jdermsci.2015.03.002

PMID:23540594

Fuertes de Vega I, Iranzo-Fernandez P, Mascaro-Galy JM

Bullous pemphigoid: clinical practice guidelines.

Actas Dermosifiliogr 105:328-46 (2014)
DOI:10.1016/j.ad.2012.10.022

PMID:26173987

Danescu S, Chiorean R, Macovei V, Sitaru C, Baican A

Role of physical factors in the pathogenesis of bullous pemphigoid: Case report series and a comprehensive review of the published work.

J Dermatol 43:134-40 (2016)
DOI:10.1111/1346-8138.13031

PMID:24434358 (description, autoantigen)

Baum S, Sakka N, Artsi O, Trau H, Barzilai A

Diagnosis and classification of autoimmune blistering diseases.

Autoimmun Rev 13:482-9 (2014)
DOI:10.1016/j.autrev.2014.01.047