Diagnostic Patterns in Pemphigus Vulgaris

By Dominik Ettlin, M.D., D.M.D.
Northwestern University Dental School
Chicago, Illinois

As most readers know, the National Pemphigus Foundation held its first annual meeting in Chicago on August 1, 1998. Attendees of the meeting were given a questionnaire. The purpose of this survey was to shed light on the diagnostic process of each participant, and to analyze the collected data for possible trends. The questionnaire was also posted on the Foundation's website for a few weeks. The collaboration between patients and clinicians is critical to gain more insight into the clinical and basic research aspects of the disease. I would like to take this opportunity to thank everyone who took the time to answer the questionnaire!

Pemphigus vulgaris is an autoimmune disorder that typically presents with sores in the mouth (oral), on the skin, or both. In clinical practice, dental and medical professionals are often challenged by the ill-defined and variable appearance of sores that present in the mouth. We were interested to answer several questions retrospectively:

1) Where does pemphigus vulgaris start: on the skin or in the mouth (oral sores)?

2) When pemphigus vulgaris starts on the skin: what is the time interval between the onset of sores and the final diagnosis? How many clinicians were consulted before the diagnosis of pemphigus vulgaris has been established?

3) Similarly, when pemphigus vulgaris starts in the mouth: what is the time interval between the onset of sores and the final diagnosis? How many clinicians (dentists or physicians) were consulted before the diagnosis of pemphigus vulgaris has been established? Thirty-seven individuals responded to the questionnaire. The analysis of the data revealed some interesting results.

Answer to question 1-In 30 individuals (81%), the first presentations of pemphigus vulgaris were mouth (oral) sores. Only 7 people or 19% experienced their first sores outside of the mouth. The observation that the disease starts in the mouth in the majority of persons confirms other reports in the medical and dental literature.

Answer to question 2-Of the 30 patients with mouth sores as the earliest disease manifestation, the time interval to diagnosis was less than 6 months for 17 patients (57%), 6-12 months for 4 patients (13%), and greater than 1 year for 9 patients (30%). By comparison, the diagnosis was established within six months in all patients (100%) experiencing skin lesions first. In other words, the proper diagnosis for the same disorder had taken different amounts of time, depending if the disease started with mouth sores or skin sores, respectively.

Answer to question 3-When the skin was the first manifestation of pemphigus vulgaris, most patients were diagnosed by one or two clinicians. This was very different for patients who experienced only mouth sores. 21 people, i.e. the majority (70%), had to consult with three or more clinicians before the early mouth sores were properly diagnosed as pemphigus vulgaris.

In summary, pemphigus vulgaris invariably affects oral tissue at some time. This study confirms previously published data that the disease typically starts in the mouth. In this sample, the diagnosis was considerably delayed in patients presenting with initial oral involvement, as compared to patients who experienced skin lesions first. More clinicians were involved before oral sores were properly diagnosed as opposed to skin lesions. Hence, health professionals should consider pemphigus vulgaris as a possible cause for mouth sores. If a physician or a dentist is unfamiliar with oral diseases, they should consider prompt and proper referral when encountering oral sores. There is a need to enhance the knowledge of clinicians regarding the oral manifestations of systemic diseases. As a further step in this direction, the data of this study will be presented at the next meeting of the International Association of Dental Research on March 11, 1999 in Vancouver, Canada

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