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Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Bullous Pemphigoid-Associated Mortality Rate in a Canadian Tertiary Referral Centre
Patrick Cyr1, Darosa Lim1, Marco Caruana1
112368 Faculty of Medicine, Université de Montréal, QC, Canada.
Journal of Cutaneous Medicine and Surgery
|April 5, 2022
Summary
Bullous pemphigoid (BP) mortality in Canada is significant, with 16.2% at 12 months and 27.6% at 24 months. Male sex, advanced age, and neurocognitive disorders predict poorer survival in BP patients.
Area of Science:
- Dermatology
- Geriatrics
- Epidemiology
Background:
- Bullous pemphigoid (BP) is the most common autoimmune blistering disease in adults, predominantly affecting those over 60.
- Global incidence is rising due to population aging, with significant, variable mortality rates reported internationally.
- Canadian population data on BP mortality and risk factors were previously lacking.
Purpose of the Study:
- To determine the 12- and 24-month overall mortality rates for Canadian patients diagnosed with bullous pemphigoid.
- To identify independent risk factors associated with adverse outcomes in this patient cohort.
Main Methods:
- A retrospective cohort study involving 166 bullous pemphigoid patients from 2010-2020 at a tertiary referral center.
- Kaplan-Meier estimator was used for cumulative mortality calculations.
- Cox proportional hazards regression model identified independent prognostic factors.
Main Results:
- The study cohort had a median age of 79.1 years, with 48.2% aged 80 or older.
- Twelve-month mortality was 16.2% (CI95% = 10.5 - 21.8) and 24-month mortality was 27.6% (CI95% = 20.5 - 34.7).
- Male sex, age 80+, and major neurocognitive disorders were independently associated with poorer overall survival.
Conclusions:
- The observed mortality rates in Canadian bullous pemphigoid patients were comparable to international findings.
- Identifying high-risk patient groups (male, elderly, neurocognitive impairment) can inform clinical management and prognosis.

