Summary

Rituximab has clearly evolved as a novel therapeutic option in refractory autoimmune bullous skin disorders such as severe pemphigus vulgaris and epidermo-lysis bullosa acquisita. The mode of action suggests that not only the production of autoantibodies is inhibited by depletion of autoreactive B cells but also the critical interaction of autoreactive T and B cells, which is essential for the perpetuation of the ongoing autoimmune response. Treatment of pemphigus with rituxi-mab has been shown to be highly effective in refractory mucocutaneous pemphigus with a prolonged biological activity due to the long-term depletion of autoreactive B cells and a lack of major side effects. In addition, single case reports suggest that TNF-a blockers maybe a therapeutic option in pemphigus and mucous membrane pemphigoid with life-threatening or therapy refractory disease course, particularly as a short-term intervention therapy. However, potential side effects due to the immunosuppressive potential of TNF-antagonists such as severe bacterial infections, viral infections and tuberculosis should be carefully considered and constitute a serious hazard in patients on chronic immunosuppressive therapy.

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