II. Epidemiology

  1. Age of onset 60 to 80 years old
  2. Men and women affected equally
  3. Incidence: 7 per 1 million in U.S.

III. Pathophysiology

  1. Autoimmune Subepidermal Blistering
  2. Mediated by IgG auto-antibodies that target basement membrane hemidesmosome at dermal-epidermal junction

IV. Causes

  1. Idiopathic (85% of cases)
  2. Specific trigger (15% of cases)
    1. Radiation Therapy
    2. Burn Injury
    3. Immunization
    4. Surgery
    5. Trauma
    6. Medications (esp. Furosemide)

V. Symptoms

  1. Pruritus
  2. Lesions may be tender at borders

VI. Signs

  1. First phase (weeks to months)
    1. Initial Urticarial, Eczematous or erythematous papular lesions
    2. Flexor creases (e.g. axilla, inguinal) are often affected
    3. Intense Pruritus
  2. Second phase
    1. Develops into large tense bullae (1 to 4 cm in diameter) after weeks to months
      1. Contain clear fluid or may at times be hemorrhagic
    2. Diffuse cutaneous involvement
      1. Often involves lower legs, Forearms, thighs, groin
      2. Also involves Abdomen
      3. Mucusa much less affected than in pemphigous vulgaris
    3. No scar formation
    4. Milia may form at previously involved sites
    5. Nikolsky Sign is absent (contrast with Pemphigus lesions)
      1. Thick walled bullae

VII. Labs

  1. Histology
    1. Subepidermal Blister
    2. Superficial dermal inflammation (Eosinophils)
  2. Immunofluorescence
    1. IgG and C3 deposition along basement membrane zone

VIII. Management

  1. First-Line
    1. Prednisone 1 mg/kg/day
    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. Leflunomide
    4. Cyclophosphamide
    5. Intravenous Immunoglobulin
    6. Dapsone
    7. Plasmapheresis
    8. Tetracycline

IX. Complications

  1. Secondary Skin Infection

X. Course

  1. Self-limited condition
  2. Chronic in many cases with exacerbations and remissions
  3. Remits with treatment by 6 years in 50% of cases

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Ontology: Bullous pemphigoid (C0030805)

Definition (NCI) An autoimmune chronic skin disorder characterized by the presence of large blisters. It usually affects elderly people and tends to subside spontaneously.
Definition (NCI) A rare autoimmune blistering skin disorder that resembles pemphigus but is histologically and clinically distinguishable by lack of evidence of acantholysis and a generally benign course.
Definition (MSH) A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis.
Concepts Disease or Syndrome (T047)
MSH D010391
ICD9 694.5
ICD10 L12.0 , L12, L12.9
SnomedCT 200912002, 156356009, 77090002, 86142006
English Pemphigoid, Pemphigoid, Bullous, Pemphigoid NOS, Pemphigoid, unspecified, Pemphigoids, pemphigoid, pemphigoid (diagnosis), bullous pemphigoid (diagnosis), bullous pemphigoid, Bullous pemphigoid NOS, Bullous Pemphigoid, Pemphigoid, Bullous [Disease/Finding], bullous pemphigoids, pemphigoids, pemphigoid bullous, Pemphigoid NOS (disorder), BP - Bullous pemphigoid, Bullous pemphigoid (disorder), Pemphigoid (disorder), bullous; pemphigoid, pemphigoid; bullous, Pemphigoid, NOS, Bullous pemphigoid
Dutch bulleus pemfigoïd, bulleus pemfigoïd NAO, bulleus; pemfigoïd, pemfigoïd; bulleus, Pemfigoïd, niet gespecificeerd, pemfigoïd, Bulleus pemfigoïd, Pemfigoïd, bulleus, Pemfigoïd
French Pemphigoïde bulleuse SAI, Pemphigoïde, Pemphigoïde bulleuse
German bulloeses Pemphigoid, bulloeses Pemphigoid NNB, Bulloeses Pemphigoid, Pemphigoidkrankheit, nicht naeher bezeichnet, Pemphigoidkrankheiten, Pemphigoid, Pemphigoid, bullöses
Italian Pemfigoide bolloso NAS, Pemfigoide, Pemfigoide bolloso
Portuguese Penfigóide bolhoso, Penfigóide bolhoso NE, Penfigoide, Penfigoide Bolhoso, Parapênfigo, Penfigóide
Spanish Penfigoide bulloso NEOM, Penfigoide bulloso, penfigoide, SAI, penfigoide, SAI (trastorno), Penfigoide Bulloso, penfigoide (trastorno), penfigoide ampolloso (trastorno), penfigoide ampolloso, penfigoide, Penfigoide, Penfigoide Ampolloso
Japanese 水疱性類天疱瘡NOS, ルイテンポウソウ, スイホウセイルイテンポウソウ, スイホウセイルイテンポウソウNOS, 水疱性類天疱瘡, 類天疱瘡-水疱性, 類天疱瘡
Swedish Pemfigoid, bullös
Czech pemfigoid bulózní, Bulózní pemfigoid, Pemfigoid, Bulózní pemfigoid NOS
Finnish Rakkulainen pemfigoidi
Korean 상세불명의 유사천포창, 유사천포창, 수포성 유사천포창
Polish Pemfigoid pęcherzowy
Hungarian Pemphigoid, Bullosus pemphigoid, Bullosus pemphigoid k.m.n.
Norwegian Bulløst pemfigoid, Pemfigoid