Please login or create an account. If you do not have access to this content, you will be shown a 30 second preview and licensing options.
- Presentation
Immunobullous Disease: Two Sides of the Atlantic
Description
The presentation focuses on the advancements and differences in the understanding and treatment of immunobullous diseases between the United States and Europe. It highlights the historical context, beginning with Louis Darwin's early work on dermatitis herpetiformis in the late 1800s, which emphasized the importance of correct diagnoses in dermatology. Major breakthroughs in immunobullous diseases emerged in the 1960s and 1980s with the discoveries of significant antigens for conditions such as pemphigoid, facilitating serum testing that enhances diagnosis accuracy. While US dermatologists often prefer biopsy over serum testing due to familiarity, European methods employ both approaches for comprehensive diagnosis. The presentation compares these practices, noting that while serum testing is popular in the US, it underutilizes the advanced European methods that incorporate thorough studies on disease mechanisms and effective treatment protocols. Additionally, advances in understanding autoimmune responses, particularly involving IgE antibodies, are discussed, with clinical trials in Europe showing promising results for treatments like dupilumab and omalizumab in refractory cases. Overall, the speaker underscores the complementary nature of findings from both sides of the Atlantic, positioning the European studies as crucial to translating discoveries into clinical practice.
View moreConclusions
- Discovery of immunobullous diseases was initially centered in the USA while clinical application and translation largely took place in Europe.
- The combination of serum testing and direct immunofluorescence (DIF) is necessary to avoid misdiagnoses in cases of pemphigoid.
- Biopsy alone is often inadequate for diagnosing pemphigoid due to its variable presentation, highlighting the need for serological testing.
- DIF achieves an accuracy of 85-90%, meaning additional serological tests are essential for a comprehensive diagnosis.
- Testing for BP180 and BP230 significantly enhances diagnostic sensitivity for pemphigoid cases that test negative by DIF.
- A multi-faceted approach to diagnosis is critical in potentially fatal conditions like pemphigoid, despite the associated costs.
- Immunologically, IgE antibodies are involved in the pathogenesis of bullous pemphigoid, indicating their clinical relevance for understanding disease mechanisms.
- Recent studies indicate that treatments targeting IgE, such as omalizumab, show promising remission rates in bullous pemphigoid patients refractory to standard therapies.
- The Bullous Disease group in Lübeck is becoming a leading center for research and clinical trials related to immunobullous diseases.
- Beutner EH, Jordon RE. Pemphigus Antibody. Proc Soc Exp Biol Med 117:505, 1964
- Meijer JM, Diercks GFH, de Lang EWG, Pas HH, Jonkman MF. Assessment of Diagnostic Strategy for Early Recognition of Bullous and Nonbullous Variants of Pemphigoid. JAMA Dermatol. 2019;155(2):158-165. doi:10.1001/jamadermatol.2018.4390
- Sardy M, Kostaki D, Varga R, Peris K, Ruzicka T. Comparative study of direct and indirect immunofluorescence and of BP 180 and 230 ELISA for diagnosis of bullous pemphigoid. J Am Acad Dermatol. 2013 Nov;69(5):748-753.
- Arch Dermatol. 2010;146: 219.
- Caux F, Patsatsi A, Karakioulaki M, Antiga E, Baselga E, Borradori L, et al. S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology. Eur Acad Dermatol Venereol. 2024 Feb 29. doi:10.1111/jdv.19880.
- Jing K, Jordan TJ, Li N, Burette S, Yang B, Marinkovich MP, et al. Anti-NC16A IgA from Patients with Linear IgA Bullous Dermatosis Induces Neutrophil-Dependent Subepidermal Blistering in Mice. J Invest Dermatol. 2024;144:24e32. doi:10.1016/j.jid.2023.05.027.
- Zone JJ, et al. IgA-TG3 Complexes in the Dermis Produce characteristic granular IgA pattern. J Immunol. 2011 Apr 1;186(7):4474-80.
- Taylor TB, Zone JJ, et al. Potassium lodide stimulates TG3 activity. Arch Biochem Biophys. 1978;112(4):555.
- Chebani R, et al. Omalizumab in the treatment of bullous pemphigoid resistant to first-line therapy: a French national multicentre retrospective study of 100 patients. Br J Dermatol. 2024 Jan 23;190(2):258-265.