Oral Pemphigus and Pemphigoid: The Role of the Dental Hygienist By Kate E. Frantz, MPH emphigus Vulgaris (PV) and Mucous Membrane Pemphigoid (MMP) are chronic autoim-mune blistering diseases that destroy proteins essential in holding together skin and mucosal cells. Autoimmune destruction of the cellular “ce-ment” results in multiple chronic ulcerations and erosions of the skin and/or mucosa. Pemphigus and pemphigoid (P/P) each affect fewer than 50,000 people in the United States, classifying these rare disorders as ultra-orphan illnesses. 1 They are not contagious and can be transmitted QHLWKHUWKURXJKWRXFKQRUE\EORRGRUÀXLGV 2 While both disorders can be effectively controlled with medication, there is no cure. Both diseases typically begin in individuals 50-60 years old, but can occur at any age. In a 2011 survey of 87 P/P patients, 63.2 percent of patients reported lesions occurred ¿UVWLQWKHPRXWK�b;ODELDODQGEXFFDOPXFRVD�f;JLQJLYDH�f; tongue or throat). 3 Lesions of PV and MMP begin as fragile, ODUJHÀXLG ¿OOHGEOLVWHUV�b;EXOODH�c;�f;ZKLFKUXSWXUHWRIRUP large, coalescing, non-healing ulcerations. PV lesions result from destruction of the cementing proteins between epi-thelial cells, and MMP lesions result from destruction of the cementing protein that anchors the overlying epithelium to the underlying connective tissue. Importantly, 80 percent of PV patients experience oral lesions before skin lesions and about 25 percent of patients have exclusively oral lesions throughout their illness. When PV affects the skin, lesions typically occur on the upper chest, back, scalp and face. 2 6LPLODUWR39�f;003OHVLRQVRFFXU¿UVWLQWKHPRXWK compared to other mucosal surfaces (ocular, genital, nasal, anal). 1 Figures 1-3 illustrate typical appearance of oral PV and MMP lesions. P Figure 1. Pemphigus Vulgaris desquamative gingivitis Diagnostic Delays Despite the very common oral involvement as the ¿UVWVLJQRIGLVHDVH�f;LWWDNHVPRUHWLPHDQGPRUHGRFWRU contacts to accurately diagnose the oral presentation of dis-HDVH2QDYHUDJH�f;33SDWLHQWVVHH¿YHGRFWRUVLQSXUVXLWRI a correct diagnosis, with 10 percent reporting seeing more than 10 health care providers. 3 It takes P/P patients an av-erage of 10 months to achieve a correct diagnosis. 3 Delayed diagnosis and ineffective treatment of P/P can increase patient pain and suffering, heighten risk for worsening illness and poor treatment response, increase medication adverse effects and risk for co-morbid disease, result in loss of productivity and reduced quality of life, cause anxiety and fear, and increase patient health care expenses. Figure 2. Pemphigus Vulgaris oral lesion The IPPF Awareness Campaign The International Pemphigus & Pemphigoid Foundation (IPPF) is dedicated to improving the quality of life of all Figure 3. Mucous Membrane Pemphigoid 16 APR 2014 access