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Bullous (& non-bullous) Pemphigoid: Evidence Based Updates and Practical Clinical Pearls

Description

This presentation discusses Bullous and Non-Bullous Pemphigoid, focusing on diagnosis, triggers, management, and emerging therapies. Bullous pemphigoid is a rare autoimmune blistering disease, with a higher prevalence in the elderly, particularly affecting women. The diagnosis relies on clinical features, histological examination of skin biopsies, and immunological tests. Non-bullous pemphigoid presents with atypical symptoms, often characterized by severe itching and varied skin lesions, with about 20% of cases displaying atypical lesions. Treatment traditionally involves systemic steroids, which must be used cautiously due to the associated risk of increased mortality in elderly patients. Emerging options, like Dupilumab and Omalizumab, show promise, but further studies are needed to assess their effectiveness compared to established therapies. The talk also highlights the impact of medications on pemphigoid incidence, particularly certain diabetes medications and checkpoint inhibitors for cancer. The evolving landscape of pemphigoid management reflects a need to balance effective treatment while minimizing risks, particularly in fragile older patients.

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Conclusions

  • Bullous pemphigoid prevalence is higher in older individuals, particularly those over 90 years old.
  • Patients with bullous pemphigoid often experience comorbidities, especially neurologic disorders like multiple sclerosis and Alzheimer's disease.
  • Diagnosis of bullous pemphigoid relies on clinical, histological, and immunological criteria, while non-bullous pemphigoid remains less defined without standardized diagnostic criteria.
  • Non-bullous pemphigoid has a significant delay in diagnosis, with patients experiencing greater pruritus and a high mortality rate.
  • Certain medications, particularly gliptins and checkpoint inhibitors, significantly increase the risk of developing bullous pemphigoid.
  • Conventional treatments often involve systemic steroids, but their long-term use is associated with increased mortality in this patient population.
  • Emerging therapies like omalizumab and dupilumab show promise in managing bullous pemphigoid, with studies indicating they can reduce steroid dependency and improve patient outcomes.
  • And emerging treatments such as JAK inhibitors are being investigated for their potential benefits in bullous pemphigoid management.
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