Clinical and immunological features of pemphigus relapse
I. Ujiie  H. Ujiie  H. Iwata  H. Shimizu
First published: 03 June 2019 https://doi.org/10.1111/bjd.17960
This summary relates to https://doi.org/10.1111/bjd.17591
British Journal of Dermatology, 180, 1498–1505, June 2019
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Summary
Pemphigus is a potentially fatal blistering skin disease. It is an autoimmune disease, meaning that the body's immune system, which normally fights off disease, in this case attacks healthy cells. The main treatments for pemphigus are with drugs called corticosteroids or immunosuppressive agents. The goal of treatment is to reach remission, a point where there are no new lesions (affected areas of skin) with minimal or no therapy (medication). However, many patients experience several relapses, meaning that after a period of improvement, the disease then worsens again, and it is often difficult for them to achieve remission. This study, from Japan, aimed to learn more about the risk factors and clinical features (signs) of pemphigus relapse. The authors retrospectively reviewed the medical records of 42 pemphigus patients. 61.9% of cases experienced relapse, usually when a medicine called oral prednisolone was tapered to around 0.1mg/kg. In a type of pemphigus called mucocutaneous pemphigus vulgaris (mcPV), the initial doses of prednisolone were lower in cases with relapse than without relapse. At relapse, mcPV shifted to subtypes (phenotypes) called mucosal dominant PV (mPV, which mainly affects the mouth and not the skin) (40%), pemphigus foliaceus (PF, affecting the skin) (20%) or others (20%). In contrast, the relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Looking at antibodies, which are produced by the immune system to help fight off disease, patients with both anti‐Dsg1 and anti‐Dsg3 antibodies to begin with, had recurrence with anti‐Dsg3 antibodies alone (40%), with both anti‐Dsg1 and Dsg3 antibodies (30%) or with anti‐Dsg1 antibody alone (20%). In conclusion, when a patient with mcPV relapses, there can be changes in the phenotype of their disease, and the antibodies being produced. At least 1mg/kg/day of prednisolone, especially for mcPV cases, and prudent tapering around 0.1mg/kg may lead to better outcomes.