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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/12/27 08:10:31」(JST)

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Pemphigoid is a group of rare autoimmune blistering skin diseases. As its name indicates, pemphigoid is similar in general appearance to pemphigus,^[1] but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells.^[2]

Pemphigoid is more common than pemphigus, and is slightly more common in women than in men. It is also more common in people over 60 years of age than it is in younger people.

Classification

IgG

The forms of pemphigoid are considered to be connective tissue autoimmune skin diseases. There are several types:

Gestational pemphigoid or Pemphigoid gestationis (PG) (formerly called Herpes gestationis)
Bullous pemphigoid (BP) Rarely affect the mouth
Mucous membrane pemphigoid, also known as Cicatricial pemphigoid (CP) (No skin involvement)

Bullous and Cicatricial pemphigoids usually affect persons who are over age 60.^[3]^[4] Gestational pemphigoid occurs during pregnancy,^[5] typically in the second or third trimester, and/or immediately following pregnancy.

IgA

Pemphigoid is usually considered to be mediated by IgG, but IgA-mediated forms have also been described.^[6]

IgA-mediated immunobullous diseases can often be difficult to treat even with usually effective medications such as rituximab.^[7]

References

^ "pemphigoid" at Dorland's Medical Dictionary
^ Pemphigoid at the US National Library of Medicine Medical Subject Headings (MeSH)
^ Cicatricial Pemphigoid at eMedicine
^ Bullous Pemphigoid at eMedicine
^ Pemphigoid Gestationis at eMedicine
^ Thomas T. Provost; John A. Flynn (2001). Cutaneous medicine: cutaneous manifestations of systemic disease. PMPH-USA. pp. 209–. ISBN 978-1-55009-100-7. Retrieved 25 June 2010.
^ He Y, Shimoda M, Ono Y, Villalobos IB, Mitra A, Konia T, Grando SA, Zone JJ, Maverakis E (2015). "Persistence of Autoreactive IgA-Secreting B Cells Despite Multiple Immunosuppressive Medications Including Rituximab". JAMA Dermatol 151 (6): 646–50. doi:10.1001/jamadermatol.2015.59. PMID 25901938.

External links

Pemphigoid diseases, Enno Schmidt and Detlef Zillikens, The Lancet, Volume 381, Issue 9863, Pages 320 - 332, 26 January 2013. doi:10.1016/S0140-6736(12)61140-4. The Lancet Seminars.
Nasal and Oral Mucosal Ulcers, Posted by Sara Fazio, NOW@NEJM, July 19th, 2013. Mucous-membrane pemphigoid. [Free]
The Canadian Pemphigus & Pemphigoid Foundation

UpToDate Contents

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1. 水疱性類天疱瘡および粘膜類天疱瘡の臨床的特徴および診断 clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid
2. 水疱性類天疱瘡のマネージメントおよび予後 management and prognosis of bullous pemphigoid
3. 水疱性類天疱瘡および粘膜類天疱瘡の疫学および病因 epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid
4. 粘膜類天疱瘡のマネージメント management of mucous membrane pemphigoid
5. 瘢痕性眼類天疱瘡 ocular cicatricial pemphigoid

English Journal

Interactions between Inflammation and Coagulation in Autoimmune and Immune-Mediated Skin Diseases.

Marzano AV, Tedeschi A, Polloni I, Crosti C, Cugno M.SourceOspedale Maggiore Policlinico, Via Pace, 9, 20122 Milano, Italy. angelovalerio.marzano@policlinico.mi.it.
Current vascular pharmacology.Curr Vasc Pharmacol.2012 Sep 1;10(5):647-52.
Inflammation and coagulation systems are simultaneously activated in autoimmune and immune-mediated skin disorders, and the cross-talk that amplifies and maintains their activation seems to have both local and systemic implications. This interplay occurs in bullous pemphigoid (BP), the prototype aut
PMID 22272909

Pathogenesis of epidermolysis bullosa acquisita, an autoimmune subepidermal bullous disease.

Hashimoto T, Ishii N, Ohata C, Furumura M.SourceDepartment of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan. hashimot@med.kurume-u.ac.jp.
The Journal of pathology.J Pathol.2012 Sep;228(1):1-7. doi: 10.1002/path.4062. Epub 2012 Jul 26.
Autoimmune bullous diseases (ABDs) are organ-specific autoimmune diseases, in which blisters on the skin and mucous membranes develop through binding of pathogenic autoantibodies to target antigens. There are two major ABD groups: the pemphigus group, showing autoantibodies to desmosomal components;
PMID 22692770

Japanese Journal

結節型類天疱瘡の1例および過去30年間の報告例の考察 (特集水疱症・膿疱症)

本多皓,綿貫沙織,安岡英美 [他]
皮膚科の臨床 54(12), 1689-1693, 2012-11
NAID 40019486282

尋常性乾癬に合併した水疱性類天疱瘡の1例 (特集水疱症・膿疱症)

岡崎静,安齋眞一,長田真一 [他]
皮膚科の臨床 54(12), 1683-1687, 2012-11
NAID 40019486254

各種局所療法や羊膜移植が有用であった粘膜類天疱瘡の3例

越後岳士,折戸秀光,濱口儒人 [他]
日本皮膚科学会雑誌 = The Japanese journal of dermatology 122(11), 2647-2654, 2012-11
NAID 40019469779

Related Pictures

★リンクテーブル★

リンク元

「類天疱瘡」

Pemphigoid
Classification and external resources
ICD-10	L12
ICD-9-CM	694.5
DiseasesDB	9760
Patient UK	Pemphigoid
MeSH	D010391

　　[★]

英: pemphigoid
関: 水疱症

自己抗体
表皮下水疱　→　緊満性水疱

表皮下水疱症(類天疱瘡群) (NDE.218)

水疱症

自己免疫性水疱症（後天性水疱症） autoimmuno blistering disease

表皮下水疱症（類天疱瘡群）

代表的な天疱瘡と類天疱瘡の比較

		尋常性天疱瘡	落葉性天疱瘡	水疱性類天疱瘡
年齢		中年～老年	中年	老年(若年もあり)
好発部位		口腔粘膜、全身	全身	全身
臨床像	皮膚所見	水疱、びらん	びらん、葉状落屑、	緊満性水疱、浮腫性紅斑、掻痒
	粘膜浸潤	＋＋	痂皮	＋
	Nikolsky現象	＋	－
病理組織像	所見	表皮内水疱 (棘融解)		表皮下水疱好酸球の浸潤
	Tzanck試験	＋	＋
	棘融解部位	表皮下層(基底細胞直上)	表皮上層(顆粒層)
抗原		Dsg3 のみ、 Dsg3 と1 の共存	Dsg1 のみ	BP180、BP230
ELISA		Dsg1(＋または－)、Dsg3(＋)	Dsg1(＋)、Dsg3(－)
蛍光抗体法所見	直接法(病変部皮膚)	表皮細胞間に IgG、C3 陽性		病変部基底膜部に IgG とC3 の線状沈着
蛍光抗体法所見	間接法(血清中)	抗表皮細胞間物質抗体(IgG)陽性		抗基底膜抗体の検出
治療		ステロイド、免疫抑制薬、血漿交換療法、γ グロブリン療法		ステロイド内服、免疫抑制薬、DDSなど

[1] "pemphigoid" at Dorland's Medical Dictionary

[2] Pemphigoid at the US National Library of Medicine Medical Subject Headings (MeSH)

[3] Cicatricial Pemphigoid at eMedicine

[4] Bullous Pemphigoid at eMedicine

[5] Pemphigoid Gestationis at eMedicine

[ProvostFlynn2001-6] Thomas T. Provost; John A. Flynn (2001). Cutaneous medicine: cutaneous manifestations of systemic disease. PMPH-USA. pp. 209–. ISBN 978-1-55009-100-7. Retrieved 25 June 2010.

[7] He Y, Shimoda M, Ono Y, Villalobos IB, Mitra A, Konia T, Grando SA, Zone JJ, Maverakis E (2015). "Persistence of Autoreactive IgA-Secreting B Cells Despite Multiple Immunosuppressive Medications Including Rituximab". JAMA Dermatol 151 (6): 646–50. doi:10.1001/jamadermatol.2015.59. PMID 25901938.

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案内

案内

pemphigoid