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Cicatricial PemphigoidAka: Scarring Pemphigoid, Mucosal Pemphigoid

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  1. See Also
    1. Pemphigoid
    2. Bullous Disease
  2. 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
  3. Pathophysiology
    1. Autoimmune bullous condition
    2. Associated conditions
      1. Stevens-Johnson Syndrome
      2. Medications
        1. Topical Glaucoma medications
        2. Practolol
        3. Clonidine
  4. 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
  5. 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
  6. 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
  7. 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)
  8. Complications
    1. Ocular Lesions may result in blindness
    2. Laryngeal stricture
    3. Esophageal Stricture
    4. Supraglottic Stenosis
  9. References
    1. Bickle (2002) Am Fam Physician 65(9):1861
    2. Cotell (2000) Am J Emerg Med 18(3):288
    3. Fleming (2000) J Am Acad Dermatol 43(4):571

Pemphigoid, Benign Mucous Membrane (C0030804)

Definition (MSH)A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring. It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement.
ConceptsDisease or Syndrome (T047)
ICD9694.6, 694.6
MSHD010390
EnglishBenign mucosal pemphigoid, Benign mucous membrane pemphigoid, Cicatricial pemphigoid, Mucosynechia atrophic bullous dermatitis, Mucosynechial atrophic bullous dermatitis, Mucous membrane pemphigoid, Scarring pemphigoid
Spanishdermatitis ampollosa mucosinequial atrofica, penfigoide benigno de las membranas mucosas, penfigoide benigno de membranas mucosas, penfigoide cicatricial, penfigoide cicatrizal, penfigoide mucoso benigno
Parent ConceptsSkin Diseases, Bullous (C0085932), Skin Diseases, Vesiculobullous (C0037275), Bullous pemphigoid (C0030805), Pemphigoid, Benign Mucous Membrane (C0030804), Disease of mucous membrane (C0151785)
SourcesDXP, ICD9CM, MSH, MTHICD9, NCI, NDFRT, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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