What is the recommended approach for mucosal management in pemphigus?

Mucosal Management in Pemphigus

Mucosal pemphigus should be managed with topical corticosteroid preparations as adjunctive therapy to systemic treatment, with betamethasone sodium phosphate 0.5 mg mouthwash (1-4 times daily) or clobetasol 0.05% ointment in Orabase as first-line topical options, while maintaining meticulous oral hygiene to prevent secondary complications. 1

Topical Corticosteroid Therapy

First-Line Topical Options

• Betamethasone sodium phosphate 0.5 mg dissolved in 10 mL of water as a 2-3 minute rinse-and-spit solution, used 1-4 times daily for widespread oral lesions 1
• Fluticasone propionate nasules diluted in 10 mL of water twice daily as an alternative mouthwash 1
• Clobetasol 0.05% ointment mixed in 50% Orabase applied twice weekly to localized lesions on dried mucosa (can be mixed by patient and refrigerated) 1

The evidence for additional benefit of topical treatments beyond systemic therapy is poor, but they are widely used in clinical practice and may help reduce systemic corticosteroid requirements 1. In mild disease confined to mucosal surfaces, topical therapy alone may suffice 1, 2.

Alternative Topical Agents

• Tacrolimus 0.1% ointment applied twice daily showed equivalent efficacy to triamcinolone acetonide 0.1% paste in a split-mouth randomized trial (n=15), with both significantly reducing mucosal involvement and pain scores 1
• Topical ciclosporin (100 mg/mL) 5 mL used three times daily demonstrated significant improvement in recalcitrant cases, though it tastes unpleasant and is expensive 1
• Intralesional triamcinolone acetonide injections may shorten time to clinical remission (126 vs 153 days) when added to conventional therapy, though this difference was not statistically significant 1

Supportive Oral Care Measures

Pain Management and Oral Hygiene

• Soft diets and soft toothbrushes to minimize local trauma 1
• Topical analgesics such as benzydamine hydrochloride 0.15% (Difflam Oral Rinse) before eating or toothbrushing 1
• Barrier preparations including Gengigel mouth rinse/gel or Gelclair for pain control 1
• Antiseptic mouthwashes (chlorhexidine gluconate 0.2%, hexetidine 0.1%, or 1:4 hydrogen peroxide solutions) to maintain oral hygiene and prevent dental decay 1

Critical pitfall: Painful gingival erosions inhibit tooth brushing, leading to plaque accumulation that compounds pain and inflammation; patients with pemphigus vulgaris have worse periodontal status than matched controls 1. Aggressive encouragement of oral hygiene despite discomfort is essential.

Infection Prevention

• Monitor and treat oral candidiasis, as patients are susceptible to fungal superinfection 1
• Regular dental follow-up to prevent complications from poor oral hygiene 1

Integration with Systemic Therapy

Mild Disease Management

• Topical corticosteroids alone can successfully control mild pemphigus confined to mucosal surfaces, with clobetasol propionate 0.05% cream achieving disease control in all 7 patients in one study, though 3 relapsed after 2-11 months 2
• Pediatric oral pemphigus vulgaris has been successfully managed with topical corticosteroid monotherapy 3

Moderate to Severe Disease

• Most patients require concomitant systemic therapy (corticosteroids with or without immunosuppressants), with topical agents serving as adjunctive treatment 1
• Prednisolone 1 mg/kg/day combined with azathioprine (up to 200 mg/day) for non-responding cases achieves complete remission in most patients, with adjuvant therapy reducing corticosteroid-related side effects 4

Clinical Monitoring

• Dilution of mouthwashes by 50% may be necessary to reduce discomfort with tacrolimus or other topical agents 1
• Topical prostaglandin E2 applied twice daily achieved complete healing by 3 months in 30% of patients with mild oral disease, though 4 of 10 patients did not improve 1

Key consideration: While evidence for topical therapy's additional benefit is limited, the British Association of Dermatologists guidelines (2017) support their use as adjunctive therapy based on widespread clinical practice and potential corticosteroid-sparing effects 1.