II. 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

III. Pathophysiology

  1. Autoimmune Bullous Condition
  2. Associated conditions
    1. Stevens-Johnson Syndrome
    2. Medications
      1. Topical Glaucoma medications
      2. Practolol
      3. Clonidine

IV. 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

V. 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

VII. 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)

VIII. Complications

  1. Ocular Lesions may result in blindness
  2. Laryngeal stricture
  3. Esophageal Stricture
  4. Supraglottic Stenosis

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Ontology: Pemphigoid, Benign Mucous Membrane (C0030804)

Definition (NCI) A chronic autoimmune disorder characterized by the development of blisters and ulcers in mucous membranes. It affects most often the gums, eyelids and genital mucosa sites.
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.
Concepts Disease or Syndrome (T047)
MSH D010390
ICD9 694.6
ICD10 L12.1
SnomedCT 200915000, 200913007, 34250006
English Benign Mucous Membrane Pemphigoid, Pemphigoid, Benign Mucous Membrane, Pemphigoid, Cicatricial, Mucous Membrane Pemphigoid, Benign, Cicatricial Pemphigoid, Benign mm pemphigoid NOS, Benign mucous membrane pemphigoid NOS, mucosynechial atrophic bullous dermatitis, cicatricial pemphigoid (diagnosis), mucosynechial atrophic bullous dermatitis (diagnosis), cicatricial pemphigoid, Pemphigoid, Benign Mucous Membrane [Disease/Finding], Benign Mucosal Pemphigoids, Mucosal Pemphigoids, Benign, Pemphigoid, Benign Mucosal, Benign Mucosal Pemphigoid, Mucosal Pemphigoid, Benign, Pemphigoids, Benign Mucosal, mucosal pemphigoid benign, mucous membrane pemphigoid, benign membrane mucous pemphigoid, Benign mucous membrane pemphigoid NOS (disorder), Cicatricial pemphigoid, Benign mucous membrane pemphigoid, Mucosynechia atrophic bullous dermatitis, Benign mucosal pemphigoid, Mucous membrane pemphigoid, Scarring pemphigoid, Benign mucous membrane pemphigoid (disorder), bullous; eczema, mucosynechial, atrophic, cicatricial; pemphigoid, eczema; bullous, mucosynechial, atrophic, pemphigoid; cicatricial, scar; pemphigoid, Mucosynechial atrophic bullous dermatitis
Dutch cicatriciaal pemfigoïd, benigne slijmvliezenpemfigoïd, bulleus; eczeem, mucosynechiaal, atrofisch, cicatricieel; pemfigoïd, eczeem; bulleus, bullosa, mucosynechiaal, atrofisch, litteken; pemfigoïd, pemfigoïd; cicatricieel, Cicatricieel pemfigoïd, Muceusmembraanpemfigoïd, benigne, Pemfigoïd, cicatricieel
German vernarbendes Pemphigoid, gutartiges Schleimhautpemphigoid, Vernarbendes Pemphigoid, Pemphigoid, benignes Schleimhaut-, Pemphigoid, narbenbildendes
Italian Pemfigoide benigno delle mucose, Pemfigoide cicatriziale, Pemfigoide benigno mucomembranoso
Portuguese Penfigóide benigno das membranas, Penfigóide cicatricial, Penfigoide Mucomembranoso Benigno, Penfigoide Cicatricial, Penfigoide Benigno de Mucosa, Penfigoide Benigno da Membrana Mucosa
Spanish Penfigoide benigno de la membrana mucosa, Pénfigo cicatricial, penfigoide benigno de las membranas mucosas, penfigoide benigno de las membranas mucosas (trastorno), dermatitis ampollosa mucosinequial atrófica, penfigoide benigno de membranas mucosas (trastorno), penfigoide benigno de membranas mucosas, penfigoide cicatricial, penfigoide cicatrizal, penfigoide mucoso benigno, Penfigoide Benigno de la Membrana Mucosa, Penfigoide Cicatricial
Swedish Pemfigoid, slemhinna, benign
Japanese ハンコンセイルイテンポウソウ, リョウセイネンマクルイテンポウソウ, 瘢痕性類天疱瘡, 類天疱瘡-良性粘膜性, 良性粘膜型類天疱瘡, 良性粘膜性類天疱瘡, 良性粘膜類天疱瘡, 類天疱瘡-瘢痕性, 類天疱瘡-良性粘膜型
Czech pemfigoid sliznice benigní, Benigní pemfigoid sliznice, Jizvící pemfigoid
Finnish Hyvänlaatuinen limakalvopemfigoidi
Russian PEMFIGOID RUBTSOVYI, PEMFIGOID DOBROKACHESTVENNYI SLIZISTYKH OBOLOCHEK, ПЕМФИГОИД ДОБРОКАЧЕСТВЕННЫЙ СЛИЗИСТЫХ ОБОЛОЧЕК, ПЕМФИГОИД РУБЦОВЫЙ
Korean 흉터성 유사천포창
Polish Pemfigoid śluzówkowy łagodny, Pemfigoid błon śluzowych łagodny
Hungarian Heges pemphigoid, Benignus nyálkahártya pemphigoid
Norwegian Slimhinnepemfigoid, benign, Benign mucosapemfigoid, Benign slimhinnepemfigoid, Pemfigoid, arrdannende, Arrdannende pemfigoid, Benign mukosal pemfigoid, Cicatrisiell pemfigoid, Pemfigoid, cicatrisiell
French Conjonctivite mucosynéchiante, Dermatite mucosynéchiante de Lortat-Jacob, Pemphigoïde bénigne des muqueuses, Pemphigoïde cicatricielle, Pemphigus oculaire