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Summary: Bullous pemphigoid (BP) is a rare, chronic skin disease that causes blistering and severe itching, mainly in elderly patients. It is an autoimmune skin disease, in which the body's own immune system is directed against structures of the skin. Specific proteins, named autoantibodies, are produced and lead to inflammation. It affects about 259 per million people in Germany. In a recent published clinical trial, two different therapies for BP were compared in 253 BP patients in the U.K. and Germany. This additional study, again from Germany and the U.K., aimed to find out if there are any markers in the blood of these patients that tell us about the course of the disease. Therefore the authors investigated the blood of 143 patients taking part in a trial called the BLISTER trial. Several tests were performed to check for the amount of autoantibodies in the blood of these patients. Afterwards the results were linked with basic parameters including age, general condition, number of blisters, side effects, and mortality. The study revealed that patients with a poor general condition had higher levels of both specific autoantibodies which cause the disease. Patients who died within one year of inclusion to the study had higher amounts of one of the specific autoantibodies. The presence of the other specific autoantibody was more frequent in older patients. In conclusion, analyzing the autoantibody profile not only helps in diagnosis, but may also be helpful in predicting the course of the disease. Linked Article: Holtsche et al. Br J Dermatol 2018; 179:918–924 [ABSTRACT FROM AUTHOR]

IgG3 is the IgG subclass with the strongest effector functions among all four IgG subclasses and the highest degree of allelic variability among all constant immunoglobulin genes. Due to its genetic position, IgG3 is often the first isotype an antibody switches to before IgG1 or IgG4. Compared with the other IgG subclasses, it has a reduced half-life which is probably connected to a decreased affinity to the neonatal Fc receptor (FcRn). However, a few allelic variants harbor an amino acid replacement of His435 to Arg that reverts the half-life of the resulting IgG3 to the same level as the other IgG subclasses. Because of its functional impact, we hypothesized that the p.Arg435His variation could be associated with susceptibility to autoantibody-mediated diseases like pemphigus vulgaris (PV) and bullous pemphigoid (BP). Using a set of samples from German, Turkish, Egyptian, and Iranian patients and controls, we were able to demonstrate a genetic association of the p.Arg435His variation with PV risk, but not with BP risk. Our results suggest a hitherto unknown role for the function of IgG3 in the pathogenesis of PV.

Treatment of recalcitrant cutaneous lupus erythematosus (CLE) is challenging. In situations where conventional treatment approaches fail mepacrine - an antimalarial/antiinflammatory drug that has fallen into oblivion in the last decades - might still be a promising option. We retrospectively analysed medical records of 10 patients with refractory CLE that were treated with mepacrine (100-200 mg/day) as mono- or combination therapy for various time intervals between 2001 and 2013 at the University Hospital Würzburg. Mepacrine was generally well tolerated. Side effects were mild and usually resolved after reduction or cessation. Over 50% of the patients experienced amelioration of their symptoms despite a previously recalcitrant clinical course. Altogether, our data demonstrate that mepacrine still remains a useful and effective therapeutic option for other-wise treatment-resistant CLE.