What is the treatment for cicatricial pemphigoid?

Treatment of Cicatricial Pemphigoid

Cicatricial pemphigoid requires systemic immunosuppressive therapy to control inflammation, prevent progression of conjunctival scarring, and preserve vision. The management approach should follow a stepladder pattern, starting with less toxic medications and escalating as needed based on disease severity and response.

Initial Assessment and Diagnosis

• Complete ocular surface examination including:

  • Examination of all mucous membranes
  • Assessment for conjunctival cicatricial changes (subepithelial fibrosis, fornix foreshortening, symblepharon)
  • Evaluation for corneal involvement (epithelial defects, vascularization, scarring)
  • Documentation with serial photography to monitor progression

• Diagnostic confirmation:

  • Conjunctival biopsy with immunofluorescence studies
  • Rule out other causes of cicatrizing conjunctivitis

Treatment Algorithm

First-Line Therapy

For mild to moderate disease:

• Dapsone 1.0 mg/kg/day (check G6PD levels before initiating) 1
• Tetracyclines (minocycline 100 mg/day or doxycycline 100 mg/day) with nicotinamide 500 mg/day 1
• Sulfapyridine 500 mg/day or sulfamethoxypyridazine 500 mg/day 1

For severe or rapidly progressive disease:

• Systemic corticosteroids (prednisone 0.5-1 mg/kg/day) as initial control, with tapering over at least 4 weeks 1
• Prompt referral to a dermatologist or ophthalmologist with expertise in immunosuppressive therapy 1

Second-Line Therapy (for inadequate response to first-line)

• Azathioprine (1-2 mg/kg/day) with normal thiopurine S-methyltransferase level 1
• Mycophenolate mofetil (500-2000 mg/day) 1
• Methotrexate (5-25 mg/week) 1

Third-Line Therapy (for refractory cases)

• Cyclophosphamide (1-2 mg/kg/day) 1, 2
• Intravenous immunoglobulin 1
• Rituximab (especially in combination with IVIG for refractory cases) 1

Adjunctive Therapies

• Aggressive treatment of dry eye:

  • Preservative-free artificial tears
  • Topical cyclosporine
  • Punctal occlusion if appropriate

• Topical corticosteroids for acute inflammation 1

  • High-potency topical steroids (clobetasol, betamethasone)
  • Use with caution due to risk of increased intraocular pressure

• Management of trichiasis and entropion to prevent corneal damage 3

Monitoring and Follow-up

• Regular monitoring for:

  • Disease activity (new lesions, progression of scarring)
  • Medication side effects
  • Ocular complications (dry eye, corneal damage, glaucoma)

• Most common treatment-related side effects to monitor 2:

  • Hematologic (anemia, leukopenia) - especially with dapsone
  • Gastrointestinal issues
  • Cardiovascular complications
  • Hepatotoxicity
  • Renal toxicity with cyclophosphamide

Treatment Goals and Outcomes

• Control of inflammation is achieved in approximately 90% of patients 2
• About 46% of patients require long-term systemic treatment to prevent recurrences 2
• Approximately one-third of patients can achieve prolonged remission off therapy 4
• Relapse occurs in about one-third of cases, requiring reinstitution of therapy 4

Important Considerations

• Early recognition and aggressive treatment are essential to prevent irreversible scarring and blindness 5
• Multiple agents are often required (32% of cases need two or more drugs) 2
• Surgical interventions for complications (symblepharon, entropion, cataract) may exacerbate inflammation and should be performed with caution, preferably when disease is in remission 3
• Long-term follow-up is mandatory as relapses can occur even after years of remission 4

The management of cicatricial pemphigoid requires a careful balance between controlling inflammation and minimizing medication side effects, particularly in elderly patients who are most commonly affected by this condition.