An Oral Disease Severity Score for pemphigus vulgaris. (1st October 2018) - Ormond, M.
Summary: Pemphigus vulgaris (PV) is a rare and often serious autoimmune disease that causes painful blistering in a number of sites including the skin, mouth, nose, throat or genitals. It does this by producing harmful antibodies that are directed to the skin or mucous membranes. The mouth is a site where the disease often starts and may continue to be affected for many years. Immune suppressant medicines are used to treat PV but in order to use these effectively and safely, clinicians need an accurate and 'reproducible' method of assessing and recording the number and site of the lesions. Reproducible means that the test can be repeated by another person and the results will be the same. Scoring tools that have been scientifically validated are therefore used and include the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and the Pemphigus Disease Area Index (PDAI). These include an oral (mouth) component but for patients with predominantly oral disease, we have devised a specific Oral Disease Severity Score (ODSS) which looks at 17 sites in the mouth, yet is quick and simple to use. In this UK study, we sought to validate ODSS and to compare its reproducibility with ABSIS, PDAI and the Physicians Global Assessment score. Ten oral medicine specialists, the majority unfamiliar with any of these methodologies, each scored 15 patients with oral PV on one day. Two clinicians rescored all 15 patients. The results have shown that the ODSS was both reliable and reproducible and was at least as good if not better than ABSIS and PDAI for assessing oral disease. By providing a more detailed method for assessing oral PV, we believe that ODSS will be more sensitive (accurate) for long term disease monitoring.