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a skin disease characterized by large thin-walled blisters (bullae) arising from normal skin or mucous membrane

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/04/02 11:06:04」(JST)

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See also: List of target antigens in pemphigus

Pemphigus vulgaris is a chronic blistering skin disease with skin lesions that are rarely pruritic, but which are often painful.^[1]^:561

Pathophysiology

See also: List of human leukocyte antigen alleles associated with cutaneous conditions

It is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 resulting in the loss of cohesion between keratinocytes in the epidermis, classified as a type II hypersensitivity reaction. It is characterized by extensive flaccid blisters and mucocutaneous erosions. The severity of the disease, as well as the mucosal lesions, is believed to be directly proportional to the levels of desmoglein 3. Milder forms of pemphigus (like foliacious and erythematoses) are more desmoglein 1 heavy. It arises most often in middle-aged or older people, usually starting with a blister that ruptures easily. The lesions can become quite extensive. The pathogenesis of the disease involves autoantibodies against desmosome proteins, separating keratinocytes from the basal layer of the epidermis. On histology, the basal keratinocytes are usually still attached to the basement membrane leading to the appearance and thus the term, "tombstoning".

Transudative fluid accumulates in between the keratinocytes and basement membrane (suprabasal split), forming a blister and resulting in what is known as a positive Nikolsky's sign. This is a contrasting feature from bullous pemphigoid, where the detachment occurs between the epidermis and dermis (subepidermal bullae).

Diagnosis

On a physical exam, pemphigus vulgaris has flat bullae and a positive Nikolsky's sign. The gold standard for diagnosis is a punch biopsy from the area around the lesion and examining it with direct immunofluorescent staining, showing acantholytic cells. These can also be seen on a Tzanck smear. These cells are basically rounded, nucleated keratinocytes formed due to antibody mediated damage to cell adhesion protein: Desmoglein.

Pemphigus vulgaris is easy to confuse with impetigo and candidiasis. IgG4 is considered pathogenic. The diagnosis can be confirmed by testing for the infections that cause these other conditions, and by a lack of response to antibiotic treatment. ^[2] Eosinophils tend to be found within the blisters and provide an important clue supporting bullous pemphigoid as the diagnosis.

Treatment

Corticosteroids and other immunosuppressive drugs are the mainstay of treatment. Based on recent studies, corticosteroids can be used in Pulse Therapy/Supra-pharmacological doses once a month to decrease Hypothalamo-pituitary axis inhibition. IVIg, rituximab, mycophenolate mofetil, methotrexate, azathioprine, and cyclophosphamide have also been used with varying degrees of success. It is a difficult disease to control.

References

^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
^ Kaplan DL (2009). "Dermclinic". Consultant 49 (3).

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1. 天疱瘡の病因、臨床症状、および診断 pathogenesis clinical manifestations and diagnosis of pemphigus
2. 尋常性天疱瘡および落葉状天疱瘡の初期マネージメント initial management of pemphigus vulgaris and pemphigus foliaceus
3. 難治性の尋常性天疱瘡および落葉状天疱瘡のマネージメント management of refractory pemphigus vulgaris and pemphigus foliaceus
4. 好酸球の生物学および好酸球増多症の原因 eosinophil biology and causes of eosinophilia
5. 皮膚の水疱を有する患者に対するアプローチ approach to the patient with cutaneous blisters

English Journal

Mediastinal follicular dendritic cell sarcoma with paraneoplastic pemphigus.

Chow SC1, Yeung EC2, Ng CSh1, Wong RH1, Fai To K3, Wan IY1.
Asian cardiovascular & thoracic annals.Asian Cardiovasc Thorac Ann.2015 Jul;23(6):732-4. doi: 10.1177/0218492314561501. Epub 2014 Nov 17.
Follicular dendritic cell sarcoma is a rare neoplasm of immune accessory cells. It occurs primarily in lymph nodes. Occurrences in the mediastinum are rarely reported. Diagnosis and management of follicular dendritic cell sarcoma remain unclear, and it is an under-recognized clinical entity. Only a
PMID 25406405

Pemphigus vegetans of the folds (intertriginous areas).

Ruocco V1, Ruocco E2, Caccavale S2, Gambardella A2, Lo Schiavo A2.
Clinics in dermatology.Clin Dermatol.2015 Jul-Aug;33(4):471-6. doi: 10.1016/j.clindermatol.2015.04.011. Epub 2015 Apr 8.
Pemphigus vegetans (P Veg), the rarest form of pemphigus, is thought to be a variant of pemphigus vulgaris (PV). Classically, two subtypes of P Veg are recognized: (1) Neumann P Veg, which usually begins as PV with vesicles and bullae that rupture to form hypertrophic granulating erosions, then evol
PMID 26051064

Hailey-Hailey disease: A fold (intertriginous) dermatosis.

Engin B1, Kutlubay Z2, Çelik U2, Serdaroğlu S2, Tüzün Y2.
Clinics in dermatology.Clin Dermatol.2015 Jul-Aug;33(4):452-5. doi: 10.1016/j.clindermatol.2015.04.006. Epub 2015 Apr 8.
Hailey-Hailey disease, also called benign familial pemphigus, is a late-onset blistering disorder that affects the flexures. There are typically painful erosions and cracks in affected areas. Lesions generally begin between 20 and 40 years of age. In two third of all cases, positive family history i
PMID 26051060

Japanese Journal

症例抗体プロファイルの変化に伴い皮膚粘膜型尋常性天疱瘡から落葉状天疱瘡へ移行した1例

岡田佳与,遠藤雄一郎,江川形平 [他]
皮膚科の臨床 56(3), 427-431, 2014-03
NAID 40020024775

口腔癌の早期発見のために

杉原一正,スギハラカズマサ,SUGIHARA Kazumasa
鹿児島大学歯学部紀要 34, 3-11, 2014
… A lesion of the oral mucosa which you should distinguish from oral cancer includes decubitus ulcer(traumatic ulcer), aphthous stomatitis, pemphigus vulgaris, oral candidiasis, oral hairy leukoplakia, papilloma. …
NAID 120005398198

症例免疫グロブリン大量療法施行時に血小板減少をきたした尋常性天疱瘡の1例

石浦信子,中島広子,浜野真紀 [他]
皮膚科の臨床 55(10), 1267-1272, 2013-10
NAID 40019835506

Related Pictures

★リンクテーブル★

リンク元	「尋常性天疱瘡」「水疱症」
関連記事	「vulgaris」

「尋常性天疱瘡」

Pemphigus vulgaris
	Classification and external resources
	; Micrograph of pemphigus vulgaris with the characteristic "tombstoning". H&E stain.
ICD-10	L10.0
OMIM	169610
DiseasesDB	9764
MedlinePlus	000882

　　[★]

ラ: pemphigus vulgaris, PV
関: 天疱瘡

概念

デスモグレイン3(Dsg-3)に対する自己抗体による自己免疫疾患
表皮内水疱
間擦部位に好発する弛緩性水疱と難治性のびらん
口腔内の水疱・びらん性病変はほぼ必発

病因

デスモグレイン3に対する自己抗体。ときにデスモグレイン1に対する自己抗体も存在する　→　皮膚粘膜型

表皮角化細胞同士を接着

病型

粘膜優位型　Dsg-3のみ　　　　の自己抗体：粘膜が冒される
粘膜皮膚型　Dsg-3に加えDsg-1の自己抗体：皮膚と粘膜の両方が冒される

疫学

中高年(40-60歳)に好発

病理

表皮基底細胞直上での棘融解水疱形成．
水疱内は少量の好酸球と棘融解細胞

症状

初発症状：口腔内水疱。ときに発熱、嘔吐、下痢、違和感、倦怠感など
水疱：表皮内水疱。弛緩性水疱。突然に健常皮面にエンドウマメ大ないし鶏卵大の水疱を生じ、数・発生部位は不定。
皮膚：Nikolsky現象陽性．

水疱病変

水疱は単房性で疱膜が薄く、緊満して内容は無菌・黄色透明であるが、のちに弛緩して混濁、ときに多少の紅暈がある。
容易に破れて湿潤性・赤色びらん面を露出し、しばしば血痂・痂皮でおおわれ、触れると疼痛がある。
一過性に色素沈着して瘢痕なく治癒するが、再発を反復して数ヵ月-数年にわたり寛解と増悪がある。

検査

病変皮膚に抗体の沈着　→　Dsg1 and/or Dsg3と反応
Tzanck検査：陽性

血液

赤沈亢進、貧血、白血球・好酸球増加、低タンパク血症、血清Na・Cl ･ Ca減少、K増加

蛍光抗体法直接法

表皮細胞間にIgG、補体の沈着

蛍光抗体法間接法

血清中に抗表皮細胞間抗体が存在

診断

治療

ステロイド、免疫抑制薬、血漿交換法、DDS、金製剤、

予後

難治