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