Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis.
BACKGROUND
Immunoapheresis/immunoadsorption is a specific tool to remove immunoglobulins and immune complexes from the circulation. Immunoapheresis is successfully used in various autoantibody-mediated diseases (such as autoimmune renal disease and others). In dermatology immunoapheresis is increasingly applied as an adjuvant treatment for severe autoimmune bullous diseases.


METHODS
We successfully employed adjuvant immunoapheresis to treat a 57-year-old man with life-threatening pemphigus vulgaris and a 30-year-old pregnant woman with severe pemphigoid gestationis. Immunoapheresis induced a rapid improvement and almost complete clearance of clinical symptoms without notable side effects. The clinical improvement was paralleled by a decline of the pathologic circulating autoantibodies. Following stabilization of his disease with immunoapheresis, the pemphigus vulgaris patient was continued on rituximab and remained almost free of symptoms for the next 12 months. The patient with pemphigoid gestationis was subsequently treated with systemic corticosteroids until the symptoms of her self-limited disease ceased.


CONCLUSIONS
Immunoapheresis might represent an excellent therapy for certain patients with severe pemphigus vulgaris or pemphigoid gestationis, unresponsive to conventional treatment regimens. We observed rapid improvement of clinical symptoms and no notable side effects.
Marker
Michael
M
Department of Dermatology, Krankenanstalt Rudolfstiftung, Vienna, Austria. michael.marker@wienkav.at
Derfler
Kurt
K
Monshi
Babak
B
Rappersberger
Klemens
K
D000328:Adult; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use*; D007167:Immunotherapy / Q000379:methods*; D008297:Male; D008875:Middle Aged; D006559:Pemphigoid Gestationis / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy*; D010392:Pemphigus / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy*; D010956:Plasmapheresis / Q000379:methods*; D011247:Pregnancy; D016896:Treatment Outcome
D007136:Immunoglobulins