Bullous Pemphigoid

1. See Also
   1. Bullous Disease
   2. Pemphigoid
2. Epidemiology
   1. Age of onset 60 to 80 years old
   2. Men and women affected equally
3. Pathophysiology
   1. Autoimmune Subepidermal Blistering
4. Symptoms
   1. Pruritus
   2. Lesions may be tender at borders
5. Signs
   1. Initial Urticarial eruption
   2. Develops into large tense bullae after weeks to months
      1. Contain clear fluid or may at times be hemorrhagic
   3. Diffuse cutaneous involvement
      1. Often involves lower legs, Forearms, thighs, groin
      2. Also involves Abdomen
      3. Mucusa much less affected than in pemphigous vulgaris
   4. No scar formation
   5. Milia may form at previously involved sites
6. Labs
   1. Histology
      1. Supepidermal Blister
      2. Superficial dermal inflammation
   2. Immunofluorescence
      1. IgG and C3 deposition along basement membrane zone
7. Management
   1. First-Line
      1. Prednisone
      2. Methotrexate may be used for prednisone intolerance
      3. Topical Corticosteroids in localized mild cases
   2. Adjunctive agents (Corticosteroid sparing)
      1. Azathioprine
      2. Mycophenolate mofetil
      3. Tetracycline
8. Course
   1. Self-limited condition
   2. Remits with treatment by 6 years in 50% of cases
9. References
   1. Bickle (2002) Am Fam Physician 65:1861-70
   2. Cotell (2000) Am J Emerg Med 18(3):288-99