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

Pulsed Intravenous Corticosteroids [Strength of Recommendation D (Gpp), Level of Evidence 4]

Pulsed intravenous corticosteroids refers to the intermittent administration of high doses of corticosteroids, usually intravenous methylprednisolone (10–20 mg kg−1 or 250–1000 mg) or equivalent doses of dexamethasone given on up to five consecutive days.[84] Generally, pulsed corticosteroids are given intravenously but they can be delivered orally.[85] The theoretical aims of 'pulsing' are to achieve more rapid and effective disease control compared with conventional oral dosing, thus allowing a reduction in long-term maintenance of corticosteroid doses and corticosteroid side-effects. These theoretical benefits have not been demonstrated conclusively.

In a well-designed, double-blind RCT, monthly oral dexamethasone pulses were of no additional benefit and were associated with more adverse effects compared with conventional oral corticosteroids and azathioprine.[76] However, this study was limited by small numbers (20 patients, 11 and nine in each arm) and a relatively short follow-up (1 year). One small, retrospective case-controlled study concluded that pulsed intravenous methylprednisolone (one course of 250–1000 mg per day for 2–5 days in eight cases; two courses in one case) resulted in increased complete remission rates (44% vs. 0%) and lower mean maintenance oral corticosteroid doses in nine patients with recalcitrant PV compared with six controls.[86] In terms of the rapidity of disease control, a retrospective case series reported signs of improvement within a week of pulsed methylprednisolone in all 12 patients,[87] but similar responses have been reported with oral corticosteroids.

Summary

There is no evidence that pulsed corticosteroids are superior to conventional oral corticosteroids for maintenance of most cases of PV. However, short-term pulsed corticosteroids could be considered in severe or recalcitrant PV to induce remission, particularly if there has been no response to high oral doses. There is no good evidence to support their use in this situation, but the personal experience of the GDG is that pulsing is very useful for rapid disease control in patients with severe disease.

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