Abstract
Pemphigus is a group of autoimmune blistering diseases of the skin and mucous membranes, which are mediated by IgG autoantibodies against desmogleins (Dsgs), cadherin type cellcell adhesion molecules in desmosomes. Oral and cutaneous blisters and erosions with acantholysis, which is defined as intraepidermal blisters due to the loss of cell-cell adhesion of keratinocytes, are typically observed. For the diagnosis of pemphigus, immunopathological findings of in vivo-bound and circulating IgG autoantibodies directed against the cell surface of keratinocytes are essential. Pemphigus has three major forms: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Patients with PV and PF have IgG autoantibodies against Dsg3 and Dsg1, respectively, while patients with PNP are characterized by autoantibodies against plakin molecules in addition to Dsgs and interface dermatitis suggesting the involvement of cell-mediated cytotoxicity. The relationship between clinical features and antibody profiles in pemphigus are logically explained by the Dsg compensation theory: The intraepithelial expression pattern of Dsg1 and Dsg3 is different between the skin and mucous membranes, and Dsg1 and Dsg3 compensate for each other when they are coexpressed in the same cell. Systemic corticosteroids are the mainstay of therapy for pemphigus, and adjuvant therapies, including immunosuppressive agents, plasmapheresis, high-dose intravenous immunoglobulin, and anti-CD20 monoclonal antibody are used for severe cases.
| Original language | English |
|---|---|
| Title of host publication | Clinical and Basic Immunodermatology |
| Subtitle of host publication | Second Edition |
| Publisher | Springer International Publishing |
| Pages | 601-612 |
| Number of pages | 12 |
| ISBN (Electronic) | 9783319297859 |
| ISBN (Print) | 9783319297835 |
| DOIs | |
| Publication status | Published - 2017 Apr 24 |
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Keywords
- Acantholysis
- Autoantibody
- Autoimmune
- Autoimmunity
- Blisters
- Desmogleins
- Desmosomes
- Intraepithelial blister
- Mucous membrane
- Paraneoplastic pemphigus
- Pemphigus
- Pemphigus foliaceus
- Pemphigus vulgaris
- Skin disease
ASJC Scopus subject areas
- Medicine(all)
- Immunology and Microbiology(all)
Cite this
Pemphigus family of disease. / Yamagami, Jun; Amagai, Masayuki.
Clinical and Basic Immunodermatology: Second Edition. Springer International Publishing, 2017. p. 601-612.Research output: Chapter in Book/Report/Conference proceeding › Chapter
}
TY - CHAP
T1 - Pemphigus family of disease
AU - Yamagami, Jun
AU - Amagai, Masayuki
PY - 2017/4/24
Y1 - 2017/4/24
N2 - Pemphigus is a group of autoimmune blistering diseases of the skin and mucous membranes, which are mediated by IgG autoantibodies against desmogleins (Dsgs), cadherin type cellcell adhesion molecules in desmosomes. Oral and cutaneous blisters and erosions with acantholysis, which is defined as intraepidermal blisters due to the loss of cell-cell adhesion of keratinocytes, are typically observed. For the diagnosis of pemphigus, immunopathological findings of in vivo-bound and circulating IgG autoantibodies directed against the cell surface of keratinocytes are essential. Pemphigus has three major forms: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Patients with PV and PF have IgG autoantibodies against Dsg3 and Dsg1, respectively, while patients with PNP are characterized by autoantibodies against plakin molecules in addition to Dsgs and interface dermatitis suggesting the involvement of cell-mediated cytotoxicity. The relationship between clinical features and antibody profiles in pemphigus are logically explained by the Dsg compensation theory: The intraepithelial expression pattern of Dsg1 and Dsg3 is different between the skin and mucous membranes, and Dsg1 and Dsg3 compensate for each other when they are coexpressed in the same cell. Systemic corticosteroids are the mainstay of therapy for pemphigus, and adjuvant therapies, including immunosuppressive agents, plasmapheresis, high-dose intravenous immunoglobulin, and anti-CD20 monoclonal antibody are used for severe cases.
AB - Pemphigus is a group of autoimmune blistering diseases of the skin and mucous membranes, which are mediated by IgG autoantibodies against desmogleins (Dsgs), cadherin type cellcell adhesion molecules in desmosomes. Oral and cutaneous blisters and erosions with acantholysis, which is defined as intraepidermal blisters due to the loss of cell-cell adhesion of keratinocytes, are typically observed. For the diagnosis of pemphigus, immunopathological findings of in vivo-bound and circulating IgG autoantibodies directed against the cell surface of keratinocytes are essential. Pemphigus has three major forms: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Patients with PV and PF have IgG autoantibodies against Dsg3 and Dsg1, respectively, while patients with PNP are characterized by autoantibodies against plakin molecules in addition to Dsgs and interface dermatitis suggesting the involvement of cell-mediated cytotoxicity. The relationship between clinical features and antibody profiles in pemphigus are logically explained by the Dsg compensation theory: The intraepithelial expression pattern of Dsg1 and Dsg3 is different between the skin and mucous membranes, and Dsg1 and Dsg3 compensate for each other when they are coexpressed in the same cell. Systemic corticosteroids are the mainstay of therapy for pemphigus, and adjuvant therapies, including immunosuppressive agents, plasmapheresis, high-dose intravenous immunoglobulin, and anti-CD20 monoclonal antibody are used for severe cases.
KW - Acantholysis
KW - Autoantibody
KW - Autoimmune
KW - Autoimmunity
KW - Blisters
KW - Desmogleins
KW - Desmosomes
KW - Intraepithelial blister
KW - Mucous membrane
KW - Paraneoplastic pemphigus
KW - Pemphigus
KW - Pemphigus foliaceus
KW - Pemphigus vulgaris
KW - Skin disease
UR - http://www.scopus.com/inward/record.url?scp=85035338314&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85035338314&partnerID=8YFLogxK
U2 - 10.1007/978-3-319-29785-9_33
DO - 10.1007/978-3-319-29785-9_33
M3 - Chapter
SN - 9783319297835
SP - 601
EP - 612
BT - Clinical and Basic Immunodermatology
PB - Springer International Publishing
ER -