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Cicatricial Pemphigoid

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Cicatricial Pemphigoid, Scarring Pemphigoid, Mucosal Pemphigoid

  • See Also
  1. Pemphigoid
  2. Bullous Disease
  • Epidemiology
  1. Incidence (rare): 1 in 12,000 to 20,000
  2. Ages affected: 60 to 80 years
  3. Gender predominence: Women by ratio of 2:1
  • Pathophysiology
  1. Autoimmune Bullous Condition
  2. Associated conditions
    1. Stevens-Johnson Syndrome
    2. Medications
      1. Topical Glaucoma medications
      2. Practolol
      3. Clonidine
  • Signs
  1. Blistering of mucus membranes
    1. Blister ruptures within hours of formation
    2. Painful erosion
    3. Most erosions heal without scarring
  2. Most common sites of involvement
    1. Oral Mucosa (most often buccal and palatal mucosa)
      1. Results in erosive Gingivitis
    2. Conjunctiva (usually bilateral)
      1. Results in chronic Conjunctivitis and scarring
  3. Other sites of involvement
    1. Skin (usually non-scarring)
    2. Scalp (Cicatricial Alopecia)
    3. Pharynx and Larynx
    4. External genitalia
    5. Nasal mucosa
    6. Anus
    7. Esophagus
  • Labs
  • Histology
  1. Subepidermal bulla
  2. Perivascular inflammatory cell infiltrates
  3. Fibrosis distinguishes from Bullous Pemphigoid
  4. Immunofluorescence with IgG, C3, IgA deposits
    1. Forms linear band at Basement Membrane Zone
  • Differential Diagnosis
  1. See Autoimmune Bullous Condition
  2. Bullous Pemphigoid
  3. Epidermolysis Bullosa Acquisita (EBA)
  4. Linear IgA Bullous Dermatosis (LABD)
  5. Systemic Lupus Erythematosus
  6. Pseudopemphigoid
  7. Pemphigus
  • Management
  • Early aggressive treatment
  1. First-Line: Prednisone 0.5 to 0.75 mg/kg/day x6 months
  2. Ocular Involvement: Azathioprine or Cyclophosphamide
  3. Skin Involvement: Topical Corticosteroids
  4. Oral Involvement
    1. Dapsone
    2. Corticosteroid gel
    3. Dexamethasone mouthwash (Roxane)
  • Complications
  1. Ocular Lesions may result in blindness
  2. Laryngeal stricture
  3. Esophageal Stricture
  4. Supraglottic Stenosis
  • References
  1. Bickle (2002) Am Fam Physician 65(9):1861-70 [PubMed]
  2. Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]
  3. Fleming (2000) J Am Acad Dermatol 43(4):571-91 [PubMed]