Should I refer a patient with bullous pemphigoid to dermatology?

Referral to Dermatology for Bullous Pemphigoid

Yes, patients with bullous pemphigoid should be referred to a dermatologist who is familiar with this condition, as they should supervise the treatment plan. 1

Rationale for Dermatology Referral

Bullous pemphigoid (BP) requires specialized care for several reasons:

• Proper diagnosis confirmation requires specialized testing including:

  • Direct immunofluorescence microscopy (DIF) - essential for diagnosis with 93% positivity rate 2
  • Skin biopsy for histopathology from early bulla 2
  • ELISA for anti-BP180 and anti-BP230 antibodies 2

• Treatment planning requires expertise to:

  • Assess disease severity and extent
  • Evaluate comorbidities and risk factors
  • Select appropriate treatment regimens
  • Monitor disease progression and treatment response

Management Considerations

Initial Evaluation

The dermatologist will:

• Confirm BP diagnosis through clinical features and laboratory investigations
• Search for risk factors and comorbidities
• Specify the type and extent of initial damage
• Evaluate age-dependent prognosis and general condition
• Consider therapeutic options 1

Treatment Options

Treatment depends on disease severity and patient comorbidities:

For Localized/Limited Disease:

1. First choice: Superpotent topical corticosteroids (clobetasol propionate 0.05%)

   • For mild disease: apply to whole body except face
   • For localized disease: apply only to lesions 1, 2

2. Second choice options:

   • Oral corticosteroids
   • Tetracycline + nicotinamide
   • Dapsone (after G6PD testing)
   • Topical immunomodulators (e.g., tacrolimus) 1

For Generalized Disease:

1. First choice:

   • Superpotent topical corticosteroids on whole body except face
   • Oral corticosteroids (prednisolone 0.5-0.75 mg/kg/day) 1, 2

2. Second choice (adjunctive therapy):

   • Azathioprine
   • Mycophenolate
   • Tetracycline + nicotinamide
   • Methotrexate
   • Chlorambucil 1

3. Third choice options for resistant cases:

   • Anti-CD20 or anti-IgE monoclonal antibodies
   • Intravenous immunoglobulins
   • Immunoadsorption
   • Plasma exchange
   • Cyclophosphamide 1

Special Considerations

Monitoring and Follow-up

Regular monitoring is essential:

• Initially every 2 weeks until disease control
• Then monthly for 3 months
• Every 2 months thereafter until treatment completion 2

Comorbidities

BP has significant associations with:

• Neurological disorders (dementia, Parkinson's disease, cerebrovascular disease)
• Cardiovascular diseases 1, 2

Multidisciplinary Care

The dermatologist may collaborate with:

• General practitioner or family physician
• Geriatrician or neurologist
• Specialized nurse
• Other healthcare professionals as needed 1

Pretherapy Screening

Before initiating treatment, the following tests should be considered:

• Complete blood count, ESR, CRP
• Renal and liver function tests
• Serology for hepatitis B, C, and HIV (if immunosuppressive therapy planned)
• Glucose-6-phosphate dehydrogenase (if dapsone treatment considered)
• Thiopurine methyltransferase (if azathioprine treatment considered) 1

Conclusion

Bullous pemphigoid is a complex autoimmune blistering disease that requires specialized dermatological expertise for proper diagnosis, treatment planning, and ongoing management. The European Dermatology Forum consensus clearly states that treatment should be supervised by a dermatologist familiar with this condition 1. Given the complexity of diagnosis, treatment options, and potential complications, referral to dermatology is essential for optimal patient outcomes.