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


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

In This Article

Topical Therapy for the Skin (Level of Evidence 1−)

PV is managed largely with systemic therapy. However, high-quality skincare is essential and adjuvant topical therapy, including topical corticosteroids, may be of additional benefit, although there are no controlled studies to confirm this. Rarely, patients with mild disease, particularly if confined to the mucosal surfaces, can be managed on topical therapy alone. Huilgol and Black have reviewed topical therapy for pemphigus and pemphigoid in detail.[229,230] Topical tacrolimus ointment 0·1% in combination with systemic treatment has been reported to heal recalcitrant facial erosions.[231] A small, randomized double-blind clinical trial (11 patients, 62 lesions) demonstrated significant benefit of pimecrolimus 1% cream over placebo for the healing of cutaneous erosions. The patients were also receiving systemic immunosuppression.[232]

Other small randomized trials treating cutaneous lesions have suggested benefit from pilocarpine gel 4%,[233] nicotinamide gel 4%[234] and epidermal growth factor (10 μg g−1) in 0·1% sulfadiazine cream.[235] Scalp lesions can be particularly persistent and are often covered in thick crust rather than being eroded. Soaking the crust in emollient or oil followed by gentle washing to remove the crust allows topical corticosteroids to penetrate better. Corticosteroid scalp preparations in an alcohol base should be avoided because they sting; lotions or creams should be used instead. Nasal lesions can be managed with topical corticosteroid nasal preparations such as fluticasone propionate nasules 400 μg twice daily.