Funding sources This work was supported by grants from the Swiss National Foundation for Scientific Research (31003A-121966 and 31003A-09811, to L.B.) and the European Community’s FP7 (Coordination Theme 1-HEALTH-F2-2008-200515, to L.B.).
EPIDEMIOLOGY AND HEALTH SERVICES RESEARCH
Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort
Article first published online: 29 OCT 2012
© 2012 The Authors. BJD © 2012 British Association of Dermatologists
British Journal of Dermatology
Volume 167, Issue 5, pages 1111–1117, November 2012
How to Cite
della Torre, R., Combescure, C., Cortés, B., Marazza, G., Beltraminelli, H., Naldi, L. and Borradori, L. (2012), Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort. British Journal of Dermatology, 167: 1111–1117. doi: 10.1111/j.1365-2133.2012.11108.x
Conflicts of interest None declared.
- Issue published online: 29 OCT 2012
- Article first published online: 29 OCT 2012
- Accepted manuscript online: 18 JUN 2012 03:18PM EST
- Accepted for publication 4 June 2012
Summary Background Prospective systematic analyses of the clinical presentation of bullous pemphigoid (BP) are lacking. Little is known about the time required for its diagnosis. Knowledge of the disease spectrum is important for diagnosis, management and inclusion of patients in therapeutic trials.
Objectives The primary aims of the study were: (i) to characterize the clinical features of BP at time of diagnosis; and (ii) to assess the diagnostic delay in BP and its impact on prognosis
Methods All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were prospectively registered by means of a standardized data collection form.
Results One hundred-seventeen patients with BP were included in the study. 97cases (82.9%) had typical features with vesicles, blisters and/or erosions at time of diagnosis, while in the remaining cases (17.1%) only excoriations, eczematous and/or urticarial infiltrated lesions were observed. Head/neck as well as palmo-plantar involvement were found in up to 20% of patients, while mucosal lesions were present in 14.5% of the cases. Diagnosis was made after a mean of 6.1 months after the first symptoms. In patients, in whom the diagnostic delay was 4 months or more (defined as late diagnosis group), lesions were more often limited to one body area. The type of lesions did not affect the diagnostic delay. Diagnosis was made more rapidly in patients with limb involvement compared to those without. The calculated mortality rate in the early and late diagnosis group was 18.9% and 17.9%, respectively, without significant difference.
Conclusion BP often presents with bullous lesions at time of diagnosis after a mean diagnostic delay of 6 months. Nevertheless, up to 20% of patients lack obvious blistering and postbullous erosions, mimicking thus a variety of inflammatory dermatoses. Localized disease is associated with an increased diagnostic delay, which has however no impact on prognosis.