Abstract
Pemphigoid diseases are a group of well defi ned autoimmune disorders that are characterised by autoantibodies against structural proteins of the dermal-epidermal junction and, clinically, by tense blisters and erosions on skin or mucous membranes close to the skin surface. The most common of these diseases is bullous pemphigoid, which mainly aff ects older people and the reported incidence of which in Europe has more than doubled in the past decade. Prognosis and treatments vary substantially between the diff erent disorders and, since clinical criteria are usually not suffi cient, direct immunofl uorescence microscopy of a perilesional biopsy specimen or serological tests are needed for exact diagnosis. In eight pemphigoid diseases the target antigens have been identifi ed molecularly, which has allowed the development of standard diagnostic assays for detection of serum autoantibodies-some of which are commercially available. In this Seminar we discuss the clinical range, diagnostic criteria, diagnostic assay systems, and treatment options for this group of diseases.
| Original language | English |
|---|---|
| Journal | The Lancet |
| Volume | 381 |
| Issue number | 9863 |
| Pages (from-to) | 320-332 |
| Number of pages | 13 |
| ISSN | 0140-6736 |
| DOIs | |
| Publication status | Published - 01.01.2013 |
Funding
We searched PubMed using the terms “pemphigoid”, “linear IgA”, “herpes gestationis”, and “epidermolysis bullosa acquisita”. Our search covered articles published in English between Jan 1, 2000, and May 31, 2012. We identified additional reports from the reference lists of selected articles. Some important older publications are cited either directly or indirectly through review articles. Contributors Both authors reviewed the scientific literature, interpreted the data, and wrote the report. ES prepared the figures. Conflicts of interest Both authors received honoraria for lectures from Roche and Fresenius Medical Care. We have received funding for research and development projects from Euroimmun (both authors), Fresenius Medical Care (DZ), Biotest (DZ), and Novartis (ES). Neither author has had, within the past 3 years, any stocks, shares, or equity in a relevant company, nor a contract of employment or named position on a company board. Acknowledgments We acknowledge those researchers whose contributions we have not cited directly, but, because of space limitations, through review articles. This work was supported by the German Research Foundation Excellence Cluster—Inflammation at Interfaces (EXC 306/1) .
