title: Plasmapheresis therapy in pemphigus vulgaris and bullous pemphigoid.
abstract: Blistering dermatitises are characterized by the presence of blisters that begin owing to acantholysis (intraepidermic blister) such as pemphigus vulgaris (PV) or owing to dermoepidermic detachment (subepidermic blister) such as bullous pemphigoid (BP). Both diseases are autoimmune pathologies characterized by the presence of autoantibodies against specific adhesion molecules of the skin and mucous membranes. PV, in which oral lesions are always present, has a progressive course that, if the disease is not treated, nearly always brings to death from sepsis within a few years. In BP, oral lesions are rare and the disease, that is most frequent in older individuals, has a chronic course with spontaneous remissions. Systemic corticosteroids and immunosuppressants are the mainstay of treatment of these two diseases. Although this therapy had reduced the mortality of the two pathologies it is associated with serious side effects. To reduce the corticosteroids dose and to improve the symptomatology in resistant therapy cases, we treated five patients with several procedures of plasma exchange. Four patients were affected by BP and one by PV. Their disease severity at onset of plasmapheresis ranged from mild to severe. One of 5 patients suffered a plasmapheresis side effect. All patients responded with complete remission of symptomatology and had a prednisone dosage reduction until 70%. Plasmapheresis is an effective treatment for PV and BP patients who have been unresponsive to conventional therapy, for those for whom conventional drugs are contraindicated, for those who show severe clinical manifestations and for those who need high doses of corticosteroids and immunosuppressants to keep the disease under control.
authors.affiliation: Servizio Immunotrasfusionale, Azienda Ospedaliera S Maria degli Angeli, Via Montereale 24, 33170 Pordenone, Italy. gustavo.mazzi@aopn.fvg.it
authors.forename: Gustavo
authors.initials: G
authors.lastname: Mazzi
authors.forename: Antonino
authors.initials: A
authors.lastname: Raineri
authors.forename: Francesco Angelo
authors.initials: FA
authors.lastname: Zanolli
authors.forename: Claudio
authors.initials: C
authors.lastname: Da Ponte
authors.forename: Dina
authors.initials: D
authors.lastname: De Roia
authors.forename: Liliana
authors.initials: L
authors.lastname: Santarossa
authors.forename: Renato
authors.initials: R
authors.lastname: Guerra
authors.forename: Bianca Maria
authors.initials: BM
authors.lastname: Orazi
mesh_terms: D000368:Aged; D003131:Combined Modality Therapy; D019468:Disease Management; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000628:therapy*; D010392:Pemphigus / Q000628:therapy*; D010951:Plasma Exchange; D010956:Plasmapheresis; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction
chemical_list: D007166:Immunosuppressive Agents; D011241:Prednisone