Abstract
Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens. Latter are also employed in various commercial ELISA systems and by immunoblotting in in-house assays available in specialized laboratories. ELISA systems are also particularly valuable for monitoring of the disease activity during the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.
Highlights
Autoimmune skin blistering diseases (AIBD) are a diverse group of dermatoses that are characterized by autoantibodies binding to antigens in the skin and mucous membranes
They can be subdivided into pemphigoid diseases (PD), with subepidermal split formation and autoantibody binding to structural components of the dermal-epidermal junction (DEJ), and pemphigus, with autoantibodies directed against desmosomal proteins that connect neighboring keratinocytes [1, 2]
Several tissues can be employed by indirect IF (IIF) microscopy to screen for serum autoantibodies in AIBD including monkey, rabbit, guinea pig, and human esophagus, monkey and rat bladder, and amnion epithelium
Summary
Edited by: Philippe Musette, Centre Hospitalier Universitaire (CHU) de Rouen, France. Reviewed by: Takashi Hashimoto, Osaka University, Japan Irina Khamaganova, Pirogov Russian National Research Medical University, Russia. Specialty section: This article was submitted to Dermatology, a section of the journal
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
