title: IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen.
abstract: Monoclonal gammopathy-associated IgA pemphigus is a debilitating skin disorder with inconsistent response to treatment. A 61-year-old woman with IgA pemphigus and monoclonal gammopathy of unknown significance had been treated unsuccessfully with cyclophosphamide/dexamethasone and then with rituximab. When the monoclonal gammopathy progressed to multiple myeloma, the patient received treatment with cyclophosphamide/doxorubicin/dexamethasone but there was no clinical response. Second-line therapy with a thalidomide/cyclophosphamide/dexamethasone combination led to severe exacerbation of the skin disorder. However, therapy with a combination regimen that included bortezomib, cyclophosphamide and dexamethasone resulted in complete and durable remission of multiple myeloma and IgA pemphigus. This suggests that bortezomib-based therapy is useful for the treatment of the rare dermatologic disorder associated with IgA gammopathy.
authors.affiliation: Department of Internal Medicine, Masaryk University, Brno, Czech Republic. z.adam@fnbrno.cz
authors.forename: Zdenek
authors.initials: Z
authors.lastname: Adam
authors.forename: Marta
authors.initials: M
authors.lastname: Krejcí
authors.forename: Ludek
authors.initials: L
authors.lastname: Pour
authors.forename: Josef
authors.initials: J
authors.lastname: Feit
authors.forename: Tomás
authors.initials: T
authors.lastname: Büchler
authors.forename: Roman
authors.initials: R
authors.lastname: Hájek
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D005260:Female; D006801:Humans; D017098:IgA Deficiency / Q000150:complications / Q000517:prevention & control*; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000188:drug therapy*; D010265:Paraproteinemias / Q000150:complications / Q000517:prevention & control*; D010392:Pemphigus / Q000150:complications / Q000517:prevention & control*; D011719:Pyrazines / Q000008:administration & dosage; D012074:Remission Induction
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide