British Association of Dermatologists' Guidelines for the Management of Pemphigus Vulgaris 2017

K.E. Harman; D. Brown; L.S. Exton; R.W. Groves; P.J. Hampton; M.F. Mohd Mustapa; J.F. Setterfield; P.D. Yesudian

Disclosures

The British Journal of Dermatology. 2017;177(5):1170-1201. 

In This Article

Oral Management (Strength of Recommendation D, Level of Evidence 3)

Oral lesions in PV are characterized by painful ulceration involving any surface of the oral cavity. The buccal mucosa, soft palate, lips and tongue are most frequently affected. Painful erosions on the gingival margins may inhibit tooth brushing resulting in an accumulation of plaque. This compounds the pain and inflammation. Furthermore, patients with PV have a worse periodontal status than seen in matched controls.[236,237]

Topical Corticosteroid Preparations

These are frequently used as adjunctive therapy. However, as most patients are on concomitant systemic therapy, evidence for the additional benefit of topical treatments is poor. Nevertheless, topical corticosteroid preparations are often used in patients with mucosal PV and include corticosteroid mouthwashes such as betamethasone sodium phosphate 0·5 mg dissolved in 10 mL of water as a 2–3-min rinse-and-spit solution one to four times a day, fluticasone propionate nasules diluted in 10 mL of water twice daily or clobetasol 0·05% ointment mixed in 50% Orabase® twice weekly applied to localized lesions on a dried mucosa. The latter can be mixed together by the patient and stored in the fridge.

Tacrolimus

In a split-mouth (two treatments compared when applied to one or other side of the mouth at the same time) randomized trial over 2 weeks (n = 15) the efficacy of triamcinolone acetonide 0·1% paste was compared with tacrolimus 0·1% ointment. The degree of mucosal involvement and pain scores were significantly reduced in both treatments compared with baseline but there was no difference between the treatments.[238] Topical tacrolimus, applied twice daily for 4 weeks, was beneficial in one case of recalcitrant PV affecting the lips.[239]

Topical Ciclosporin

There are small numbers of reports indicating that topical ciclosporin is effective for the oral lesions of PV. A 5-mL (500-mg) oral suspension used three times a day for 2 months in oral pemphigus (n = 12) recalcitrant to conventional treatment was reported to result in significant improvement in both symptoms and signs of PV.[240]

Ciclosporin mouthwash (100 mg mL−1) 5 mL used three times per day was effective within 6 months in a patient with recalcitrant oral lesions for 20 years.[241] Treatment was reduced and the patient was maintained on a once-daily mouthwash for 5 years. In a further three patients with PV, 67% (two of three) had a clinical improvement.[242] However, topical ciclosporin tastes unpleasant and is relatively expensive.[242]

Intralesional Triamcinolone

Mignogna et al. evaluated the efficacy of perilesional/intralesional triamcinolone acetonide injections in oral PV in addition to conventional immunosuppressive therapy plus topical corticosteroid (n = 16) in an open-label trial.[243] In comparison with a group of patients not receiving injections (n = 19), the perilesional/intralesional triamcinolone acetonide group achieved a shorter time to clinical remission (126 vs. 153 days; not statistically significant) and obtained acceptable compliance with this treatment.

Topical Prostaglandin E2

Topical prostaglandin E2 applied twice daily in 10 patients with oral lesions in PV resulted in complete healing by 3 months in 30% of patients with PV (three of 10). They had mainly mild disease affecting one mucosal site. A further three patients improved as long as treatment was continued, but relapsed within 7–10 days of stopping therapy, while four of 10 did not improve. Other treatments had been discontinued 2 weeks prior to the study.[244]

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