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Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study

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Summary Background 

Bullous pemphigoid (BP), pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune bullous diseases characterized by the presence of tissue-bound and circulating autoantibodies directed against disease-specific target antigens of the skin. Although rare, these diseases run a chronic course and are associated with significant morbidity and mortality. There are few prospective data on gender- and age-specific incidence of these disorders. Objectives 

Our aims were: (i) to evaluate the incidence of BP and PV/PF in Swiss patients, as the primary endpoint; and (ii) to assess the profile of the patients, particularly for comorbidities and medications, as the secondary endpoint. Methods 

The protocol of the study was distributed to all dermatology clinics, immunopathology laboratories and practising dermatologists in Switzerland. All newly diagnosed cases of BP and pemphigus occurring between 1 January 2001 and 31 December 2002 were collected. In total, 168 patients (73 men and 95 women) with these autoimmune bullous diseases, with a diagnosis based on clinical, histological and immunopathological criteria, were finally included. Results 

BP showed a mean incidence of 12·1 new cases per million people per year. Its incidence increased significantly after the age of 70 years, with a maximal value after the age of 90 years. The female/male ratio was 1·3. The age-standardized incidence of BP using the European population as reference was, however, lower, with 6·8 new cases per million people per year, reflecting the ageing of the Swiss population. In contrast, both PV and PF were less frequent. Their combined mean incidence was 0·6 new cases per million people per year. Conclusions 

This is the first comprehensive prospective study analysing the incidence of autoimmune bullous diseases in an entire country. Our patient cohort is large enough to establish BP as the most frequent autoimmune bullous disease. Its incidence rate appears higher compared with other previous studies, most likely because of the demographic characteristics of the Swiss population. Nevertheless, based on its potentially misleading presentations, it is possible that the real incidence rate of BP is still underestimated. Based on its significant incidence in the elderly population, BP should deserve more public health concern.
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Keywords: Switzerland; autoimmune bullous diseases; bullous pemphigoid; epidemiology; incidence; pemphigus

Document Type: Research Article

Affiliations: 1: Service de Dermatologie, Hôpitaux Universitaires de Genève, 1211 Geneva 14, Switzerland 2: Universitätsklinik für Dermatologie, Inselspital, 3010 Bern, Switzerland 3: Universitätsklinik für Dermatologie, Universitätsspital, 8091 Zurich, Switzerland 4: Service de Dermatologie, CHUV, 1011 Lausanne, Switzerland 5: Universitätklinik für Dermatologie, Kantonspital, 4031 Basel, Switzerland 6: Dermatologisches Ambulatorium, Stadtspital Triemli, 8004 Zurich, Switzerland 7: Servizio di Dermatologia, Ospedali Riuniti, 24128 Bergamo, Italy

Publication date: 01 October 2009

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