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

Follow-up and Tapering of Treatment

Once remission is induced, there should follow a period of maintenance treatment using the minimum drug doses required for disease control and during which occasional blisters are acceptable. Drug doses should be reduced slowly (see section 12) and patients should remain under follow-up while they remain on therapy. Ultimately, treatment may be withdrawn if there has been prolonged clinical remission. The chances of relapse are reduced if immunofluorescence or ELISA studies are negative, for example the risk of relapse is 13–46% if DIF is negative, 44–100% if DIF is positive, 24% if IIF is negative, 57% if IIF is positive,[282–284] 25% if desmoglein 3 ELISA is negative and 56% if desmoglein 3 ELISA is positive.[285]

In DIF-negative patients, there is some evidence to suggest that relapse is less likely the longer a patient has been in remission on minimal therapy prior to stopping treatment: 46% in all DIF-negative patients, 22% in those in remission for 6 months and 0% with remission of over 12 months.[284] However, DIF can remain positive occasionally in patients who are in remission and off all treatment.[21] A less invasive and relatively simple alternative to DIF on a skin biopsy, in this situation, is DIF on the outer root sheath of plucked hairs.[286] However, this investigation is not widely available at present.

There is no evidence to guide the order in which treatments are reduced and withdrawn in PV. However, it is common practice to withdraw corticosteroids first,[287,288] to minimize their side-effects, while maintaining adjuvant immunosuppressants at full dose (see section 12 for guidance on the rate of dose reduction). Thereafter, adjuvant drugs can be tapered slowly if remission is maintained. If complete treatment withdrawal is successful, and the patient remains in complete remission for a prolonged period, discharge to their primary-care physician is reasonable, but patients and their carers should be warned that PV can recur, in which case they should be referred to secondary care immediately.

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