Pemphigus diseases (overview)L10.-

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 27.01.2024

Dieser Artikel auf Deutsch

Synonym(s)

Bladder Dysfunction

Requires free registration (medical professionals only)

Please login to access all articles, images, and functions.

Our content is available exclusively to medical professionals. If you have already registered, please login. If you haven't, you can register for free (medical professionals only).


Requires free registration (medical professionals only)

Please complete your registration to access all articles and images.

To gain access, you must complete your registration. You either haven't confirmed your e-mail address or we still need proof that you are a member of the medical profession.

Finish your registration now

DefinitionThis section has been translated automatically.

Group of chronic blistering autoimmune diseases with intraepidermal blistering of the skin and mucous membranes, characterized as follows:

  • Clinically characterized by flaccid, easily injured blisters and an eminently chronic course.
  • Histologically characterized by intraepidermal acantholytic blistering due to loss of cell-to-cell adhesion of keratinocytes.
  • Immunohistopathologically by tissue-bound and circulating autoantibodies of the IgG (and IgA) class against surface epitopes of keratinocytes.
  • The functional inhibition of desmogleins by pemphigus antibodies leads to a loss of adhesion of the keratinocytes and to intraepidermal blister formation.

ClassificationThis section has been translated automatically.

The group of pemphigus diseases can be divided into:

EtiopathogenesisThis section has been translated automatically.

Autoimmune disease with formation of circulating autoantibodies against desmosomal adhesion molecules (desmogleins - more rarely desmocollins) of the cadherin group (calcium-dependent, transmembranous adhesion molecules), which are responsible for the intercellular cohesion of the epithelia. In classic pemphigus vulgaris, the binding of autoantibodies to the extracellular domain of the desmogleins leads to dissociation of the intercellular connections(desmosomes) without major inflammatory symptoms (no involvement of inflammatory cells or proteases!) and thus to acantholysis with degeneration of the keratinocytes ( Tzanck cell). The antigens of pemphigus vulgaris and foliaceus are glycoproteins of 130 and 160 kDa. The 160 kDa antigen corresponds to desmoglein 1 and characterizes pemphigus foliaceus.

DiagnosisThis section has been translated automatically.

Histology, direct immunohistology (DIF) from perilesional skin with detection of keratinocyte-bound autoantibodies. Detection of circulating autoantibodies.

Differential diagnosisThis section has been translated automatically.

Blistering diseases of other genesis.

TherapyThis section has been translated automatically.

S.u. and the respective clinical forms.

General therapyThis section has been translated automatically.

See below the respective clinical pictures.

TablesThis section has been translated automatically.

Immunological constellations

Disease

Autoantibodies

Autoantigen

pemphigus vulgaris

(Mucosa type and mucocutaneous type)

IgG

Desmoglein 3

desmoglein 1

Desmocolline

Plakoglobin

Cholinergic receptor of keratinocytes

pemphigus foliaceus

IgG

desmoglein 1

Plakoglobin

Desmoglein 3

Desmoplakin 1

Desmoplakin 2

Cholinergic receptor of keratinocytes

pemphigus vegetans

IgG

Desmoglein 3

desmoglein 1

desmocollin 1

desmocollin 2

Pemphigus herpetiformis

IgG

Desmoglein 1 and 3

Desmocollin 3

pemphigus erythematosus

IgG

desmoglein 1

Nuclear antigens

paraneoplastic pemphigus

IgG

Desmoglein 3

Desmocollin 1.3 and 3

Plakophilin

Desmoplakin 1 and 2

Evoplakin

alpha-2-macroglobulin -like protein

BP 230, Periplakin

Plectin

Desmoglein

Pemphigus drug-induced

IgG

desmoglein 1

Desmoglein 3

IgA pemphigus (type: subcorneal pustular dermatosis)

IgA

desmocollin 1

IgA pemphigus (type: intraepidermal neutrophil IgA dermatosis)

IgA

Desmoglein 1 and 3

LiteratureThis section has been translated automatically.

  1. Alpsoy E et al. (2015) Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res PubMed PMID: 25589418.
  2. Hashimoto T et al. (2018) Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis. Front Immunol 9:994.

  3. Ishii K et al.(2015) Importance of serological tests in diagnosis of autoimmune blistering diseases. J Dermatol doi: 10.1111/1346-8138.12703

Authors

Last updated on: 27.01.2024