title: [Treatment-refractory anti-laminin 332 mucous membrane pemphigoid. Remission following adjuvant immunoadsorption and rituximab].
abstract: Mucous membrane pemphigoid (MMP) is clinically characterized by predominant involvement of mucous membranes which in case of conjunctival lesions can lead to blindness. In MMP, autoantibodies are directed against different proteins of the dermal-epidermal junction; in 25% of cases, laminin 332 is the target. Anti-laminin 332 MMP with ocular involvement is particularly difficult to treat. A 46-year-old Caucasian man with anti-laminin 332 pemphigoid and extensive oral and nasal erosions as well as severe conjunctival involvement did not respond to intravenous dexamethasone-cyclophosphamide pulses combined with oral cyclophosphamide. After initiation of a therapeutic regimen originally established for the treatment of pemphigus, including immunoapheresis and rituximab in combination with intravenous dexamethasone-cyclophosphamide pulses and oral mycophenolate mofetil, lesions cleared within 4 months and circulating autoantibody levels became undetectable 3 months later. This is the first report of the successful use of adjuvant immunoapheresis and rituximab in previously treatment-refractory anti-laminin 332 MMP.
authors.affiliation: Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Lübeck.
authors.forename: A
authors.initials: A
authors.lastname: Recke
authors.forename: I
authors.initials: I
authors.lastname: Shimanovich
authors.forename: P
authors.initials: P
authors.lastname: Steven
authors.forename: L
authors.initials: L
authors.lastname: Westermann
authors.forename: D
authors.initials: D
authors.lastname: Zillikens
authors.forename: E
authors.initials: E
authors.lastname: Schmidt
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D001323:Autoantibodies / Q000276:immunology* / Q000302:isolation & purification; D015815:Cell Adhesion Molecules / Q000276:immunology*; D003131:Combined Modality Therapy; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000276:immunology* / Q000628:therapy*; D012074:Remission Induction; D000069283:Rituximab; D017211:Treatment Failure; D016896:Treatment Outcome; D000097794:Kalinin
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D007166:Immunosuppressive Agents; D000069283:Rituximab; D000097794:Kalinin