Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Search research articles

Related Experiment Videos

Bullous and cicatricial pemphigoid.

G J Anhalt¹, L H Morrison

¹Johns Hopkins University, Department of Dermatology, Baltimore, Maryland 21205.

Journal of Autoimmunity

|February 1, 1991

Summary

Bullous pemphigoid (BP) is an autoimmune blistering disease. Autoantibodies target hemidesmosome proteins, causing skin blistering, though definitive proof of this mechanism is still incomplete.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Decreased recognition of paraneoplastic pemphigus in patients previously treated with anti-CD 20 monoclonal antibodies.

The British journal of dermatology·2019

Same author

Efficacy of intravenous immunoglobulin (IVIG) affinity-purified anti-desmoglein anti-idiotypic antibodies in the treatment of an experimental model of pemphigus vulgaris.

Clinical and experimental immunology·2010

Same author

Treatment of pyoderma gangrenosum with intravenous immunoglobulin.

The British journal of dermatology·2007

Same author

Pernicious anemia and widespread absence of gastrointestinal endocrine cells in a patient with autoimmune polyglandular syndrome type I and malabsorption.

The Journal of clinical endocrinology and metabolism·2006

Same author

Protective effect of intravenous immunoglobulin (IVIG) in an experimental model of pemphigus vulgaris.

Clinical and experimental immunology·2005

Same author

Paraneoplastic pemphigus in a dog with splenic sarcoma.

Veterinary pathology·2005

Area of Science:

Dermatology
Immunology
Cell Biology

Background:

Bullous pemphigoid (BP) and cicatricial pemphigoid are autoimmune blistering diseases.
These conditions involve epithelial detachment from the stroma, with IgG and complement deposition at the lamina lucida.
The primary immune response involves IgG4 subclass autoantibodies targeting hemidesmosome proteins.

Purpose of the Study:

To investigate the role of specific antigens in bullous pemphigoid pathogenesis.
To explore the function of 230 kD and 180 kD transmembrane proteins in epithelial adhesion.

Main Methods:

Analysis of antibody responses in affected tissues.
Identification and characterization of antigens involved in blister formation.
Review of recent studies defining antigen sequences and cDNA encoding.

Main Results:

Autoantibodies in BP target unique 230 kD and 180 kD hemidesmosome proteins.
These antigens are crucial for stratified squamous epithelial cell adhesion.
Recent structural data provides insights into antigen function.

Conclusions:

Bullous pemphigoid is considered an autoimmune disease mediated by antihemidesmosome autoantibodies.
The binding of these autoantibodies to specific antigens is strongly suspected to cause cutaneous lesions.
Further research is needed to definitively prove the causative role of these autoantibodies in BP pathogenesis.

Related Experiment Videos