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Bullous Pemphigoid
Aka: Bullous Pemphigoid
- See Also
- Bullous Disease
- Pemphigoid
- Epidemiology
- Age of onset 60 to 80 years old
- Men and women affected equally
- Incidence: 7 per 1 million in U.S.
- Pathophysiology
- Autoimmune Subepidermal Blistering
- Mediated by IgG auto-antibodies that target basement membrane hemidesmosome at dermal-epidermal junction
- Causes
- Idiopathic (85% of cases)
- Specific trigger (15% of cases)
- Radiation Therapy
- Burn Injury
- Immunization
- Surgery
- Trauma
- Medications (esp. Furosemide)
- Symptoms
- Pruritus
- Lesions may be tender at borders
- Signs
- First phase (months)
- Initial Urticarial or erythematous lesions
- Flexor creases (e.g. axilla, inguinal) are often affected
- Intense Pruritus
- Second phase
- Develops into large tense bullae after weeks to months
- Contain clear fluid or may at times be hemorrhagic
- Diffuse cutaneous involvement
- Often involves lower legs, Forearms, thighs, groin
- Also involves Abdomen
- Mucusa much less affected than in pemphigous vulgaris
- No scar formation
- Milia may form at previously involved sites
- Nikolsky Sign is absent (contrast with Pemphigus lesions)
- Thick walled bullae
- Labs
- Histology
- Subepidermal Blister
- Superficial dermal inflammation (Eosinophils)
- Immunofluorescence
- IgG and C3 deposition along basement membrane zone
- Management
- First-Line
- Prednisone 1 mg/kg/day
- Methotrexate may be used for Prednisone intolerance
- Topical Corticosteroids in localized mild cases
- Adjunctive agents (Corticosteroid sparing)
- Azathioprine
- Mycophenolate mofetil
- Leflunomide
- Cyclophosphamide
- Intravenous Immunoglobulin
- Dapsone
- Plasmapheresis
- Tetracycline
- Complications
- Secondary Skin Infection
- Course
- Self-limited condition
- Chronic in many cases with exacerbations and remissions
- Remits with treatment by 6 years in 50% of cases
- References
- Long (2016) Crit Dec Emerg Med 30(7):3-10
- Bickle (2002) Am Fam Physician 65:1861-70 [PubMed]
- Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]