How do you manage pemphigus wounds, including immunosuppressive therapy and wound care?

Management of Pemphigus Wounds

For pemphigus wounds, initiate systemic immunosuppression with oral prednisolone 1-1.5 mg/kg/day combined with azathioprine 2-3 mg/kg/day or mycophenolate mofetil 2-3 g/day, while simultaneously implementing meticulous local wound care that preserves blister roofs and uses non-adherent dressings with daily antimicrobial cleansing. 1

Systemic Immunosuppressive Therapy

First-Line Treatment

• Oral corticosteroids are the cornerstone of therapy, with prednisolone 1-1.5 mg/kg/day as the standard initial dose for pemphigus vulgaris 1
• Add adjuvant immunosuppression from the start rather than using corticosteroids alone, as this provides superior steroid-sparing effects and reduces long-term corticosteroid toxicity 1
• Azathioprine 2-3 mg/kg/day is the preferred first-line adjuvant, with the caveat that clinical effect requires 6-8 weeks to manifest 1
• Mycophenolate mofetil 2-3 g/day in divided doses is an equally effective alternative to azathioprine, particularly in patients with thiopurine methyltransferase deficiency or azathioprine intolerance 1

Severe or Refractory Disease

• For life-threatening disease or failure to respond within 1-3 weeks, escalate to pulsed intravenous methylprednisolone 250-1000 mg daily for 1-3 days 1
• Rituximab 2 x 1000 mg infusions separated by 2 weeks should be considered as third-line therapy, achieving 89% complete remission at 2 years 1, 2
• Rituximab is now approved as first-line therapy for moderate-to-severe pemphigus when combined with short-term corticosteroids, based on superior efficacy and safety compared to corticosteroid monotherapy 2, 3

Tapering Strategy

• Reduce prednisolone by 5-10 mg weekly initially, then taper more slowly below 20 mg daily, with a goal of ≤10 mg daily while continuing adjuvant immunosuppression 1
• Withdraw corticosteroids first to minimize their side-effects, while maintaining adjuvant immunosuppressants at full dose 1
• Do not attempt dose reduction until disease control is achieved, defined as no new blisters and healing of existing lesions 1

Local Wound Care

Blister Management

• Never deroof intact blisters, as the blister roof acts as a natural biological dressing that reduces infection risk and promotes re-epithelialization 1, 4
• For large or functionally problematic blisters, pierce at the base with a sterile needle (bevel facing up) to facilitate gravity drainage, then apply gentle pressure with sterile gauze to absorb fluid 1, 4
• Select the puncture site where fluid will drain by gravity to discourage refilling 1

Cleansing Protocol

• Gently cleanse blisters and erosions with antimicrobial solution before and after drainage, taking care not to cause further trauma 1, 4
• Implement daily washing with antibacterial products to decrease bacterial colonization and reduce infection risk 1, 4
• For extensive erosions, consider antiseptic baths with potassium permanganate or antiseptic-containing bath oils (e.g., Dermol Plus or Oilatum) for a few days to dry lesions and prevent infection 1

Dressing Application

• Cover painful eroded or raw areas with low-adhesion, non-adherent dressings such as Mepitel or Atrauman, held in place with soft elasticated viscose 1, 4
• Change dressings using aseptic technique to minimize infection risk 1, 4
• Apply petrolatum-based products or petrolatum-based antibiotic ointment to support barrier function and accelerate healing 4

Infection Prevention and Monitoring

Surveillance

• Monitor daily for signs of infection, including increased erythema, purulent discharge, fever, or worsening pain 1, 4
• Infection and sepsis are major causes of mortality in pemphigus, requiring heightened vigilance especially with escalated immunosuppression 1, 5
• Obtain bacterial and viral swabs from erosions showing clinical signs of infection 1

Infection Management

• Use systemic antibiotics if there are local or systemic signs of infection or extending skin infection, guided by local antibiotic policy 1
• Topical antimicrobials may be applied for short periods only 1
• Barrier nurse patients with extensive erosions to reduce nosocomial infection risk 1

Pain Management

• Provide adequate analgesia for both acute and maintenance (background) pain, with ability to provide timely additional short-term boosts during dressing changes 1
• Offer analgesia prior to blister care procedures, as many patients report pain or burning sensation during wound management 1
• Consider pain team consultation for patients with extensive disease 1

Oral Mucosal Involvement

Topical Oral Therapy

• Use soft diets and soft toothbrushes to minimize local trauma to oral mucosa 1
• Apply topical analgesics or anesthetics such as benzydamine hydrochloride 0.15% (Difflam Oral Rinse) prior to eating or toothbrushing 1
• Maintain rigorous oral hygiene with antiseptic mouthwashes such as chlorhexidine gluconate 0.2% (Corsodyl), hexetidine 0.1% (Oraldene), or 1:4 hydrogen peroxide solutions 1
• For multiple oral erosions, use soluble betamethasone sodium phosphate 0.5 mg tablet dissolved in 10 mL water up to four times daily, holding the solution in the mouth for about 5 minutes 1

Common Pitfalls to Avoid

• Do not use corticosteroids as monotherapy without adjuvant immunosuppression, as this increases cumulative corticosteroid exposure and associated toxicity 1
• Do not remove blister roofs, as this increases infection risk and delays healing 1, 4
• Do not delay escalation of therapy if disease control is not achieved within 1-3 weeks of initial treatment 1
• Do not overlook infection surveillance, as sepsis remains a leading cause of death in pemphigus patients 1, 5
• Do not taper immunosuppression too rapidly, as relapse rates are high; occasional blisters during maintenance are acceptable and do not mandate dose escalation 1