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

Plasma Exchange/Plasmapheresis (Strength of Recommendation D, Level of Evidence 3)

Plasma exchange has been used for many years in the management of antibody-mediated autoimmune disease, including pemphigus. Thus, multiple case reports and small case series have reported clinical benefit, short-term falls in IIF titres and a corticosteroid-sparing effect of plasma exchange.[196–208] In general, these were problematic patients with either corticosteroid side-effects, poorly controlled disease on conventional therapy or life-threatening disease. However, a randomized study of patients with newly diagnosed pemphigus treated with oral corticosteroids with or without additional plasma exchanges failed to demonstrate any additional clinical benefit of plasma exchange. Cumulative corticosteroid doses and changes in IIF titre in the two groups were similar. Furthermore, there were four deaths from sepsis in the plasma exchange group.[209]

In the cases reported that have been treated successfully, plasma exchange has been combined with both corticosteroids and immunosuppressive drugs – it is thought that the latter are necessary for sustained clinical effect in order to prevent rebound production of autoantibodies stimulated by the plasma exchange.[196,199,204,205,210–213] IVIg has been reported to have a similar action and has been used successfully in combination with plasmapheresis.[214]

Plasma exchange is not without adverse effects as, in addition to pathogenic immunoglobulins, other important plasma proteins are removed such as clotting factors that can result in coagulation defects on removal.[215]

Summary

Plasma exchange cannot be recommended as a routine treatment option in newly presenting patients with pemphigus but may be considered in refractory cases if combined with corticosteroids and immunosuppressant drugs.

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