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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）』「2016/04/22 01:28:26」(JST)

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Pemphigus foliaceus is an autoimmune blistering disease of the skin with characteristic lesions that are scaly, crusted erosions, often on an erythematous base.^[1] Mucosal involvement is absent even with widespread disease.^[2]

If there is an autoimmune IgG buildup in the epidermis, then nearly almost all of the antibodies are aimed against desmoglein 1. The effect of the antibodies and the immunological pathway is most likely either of three mechanisms:

Steric hindrance of the desmoglein 1: The antibody caps off the site for intracellular binding to another keratinocyte.
Activation of an endocytic pathway: The antibody activates a pathway which causes an internalization of desmogleïn 1, which in turn causes a loss of adhesion.
Disruption of function: In this case, the antibody blocks the desmoglein 1 from being formed into a desmosome. This in turn causes a loss of adhesion with acantholysis as a result.

Distribution

Pemphigus is endemic in the rural areas of Brazil, especially along inland riverbeds.^[3]

Summary

The National Institute of Arthritis and Musculoskeletal and Skin Diseases describes it like this:

Normally, our immune system produces antibodies that attack viruses and harmful bacteria to keep us healthy. In people with pemphigus, however, the immune system mistakenly attacks the cells in the epidermis, or top layer of the skin, and the mucous membranes. The immune system produces antibodies against proteins in the skin known as desmogleins. These proteins form the glue that keeps skin cells attached and the skin intact. When desmogleins are attacked, skin cells separate from each other and fluid can collect between the layers of skin, forming blisters that do not heal. In some cases, these blisters can cover a large area of skin.^[4]

History

Pierre Louis Alphee Cazenave first described the disease in 1844.^[5]

References

^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 558-562. McGraw-Hill. ISBN 0-07-138076-0.
^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). Page 558. McGraw-Hill. ISBN 0071380760.
^ NIH Publication No. 15–7083 (June 2015). "Questions and Answers about Pemphigus". National Institute of Arthritis and Musculoskeletal and Skin Disease. NIH. Retrieved 23 October 2015.
^ Medscape Reference

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1. 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
2. 天疱瘡の病因、臨床症状、および診断 pathogenesis clinical manifestations and diagnosis of pemphigus
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. 頭皮の発疹を有する患者へのアプローチ approach to the patient with a scalp disorder
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

Distinctive Treg associated CCR4-CCL22 expression profile with altered frequency of Th17/Treg cell in the immunopathogenesis of Pemphigus Vulgaris.

Asothai R1, Anand V1, Das D1, Antil PS1, Khandpur S2, Sharma VK2, Sharma A3.
Immunobiology.Immunobiology.2015 Oct;220(10):1129-35. doi: 10.1016/j.imbio.2015.06.008. Epub 2015 Jun 17.
Pemphigus Vulgaris (PV), a relatively common autoimmune blistering disease in India, primarily mediated by anti-Desmoglein 3 (anti-Dsg3) autoantibodies. T-helper 17 (Th17) and T-regulatory (Treg) cells play significant role in regulating immune homeostasis in autoimmune disorders. To understand immu
PMID 26093920

Oral glucocorticoid pulse therapy for induction of treatment of canine pemphigus foliaceus - a comparative study.

Bizikova P1,2, Olivry T1,2.
Veterinary dermatology.Vet Dermatol.2015 Oct;26(5):354-e77. doi: 10.1111/vde.12241. Epub 2015 Aug 6.
BACKGROUND: The management of canine pemphigus foliaceus (PF) often requires long-term immunosuppressive treatment that is often associated with unacceptable adverse effects. High-dose glucocorticoid pulse therapy, an alternative protocole used for pemphigus in people, has been shown to provide rapi
PMID 26248494