Please activate JavaScript!
Please install Adobe Flash Player, click here for download
ePaper created 2014-09-15, 12:31:02 | version 1.32.01
References 1. Jong MC de, Bruins S, Heeres K, et al. Bullous pemphigoid and epidermolysis bullosa acquisita. Differentiation by fluorescence overlay antigen mapping. Arch Dermatol. 1996; 132: 151-7. 2. Vodegel RM, de Jong MC, Pas HH, et al. Anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita: differentiation by use of indirect immunofluorescence microscopy. J Am Acad Dermatol. 2003; 48: 542-7. 3. Vodegel RM, Kiss M, De Jong MC, et al. The use of skin substrates deficient in basement membrane molecules for the diagnosis of subepidermal autoimmune bullous disease. Eur J Dermatol 1998; 8: 83-5. 4. Vodegel RM, de Jong MC, Meijer HJ, et al. Enhanced diagnostic immunofluorescence using biopsies transported in saline. BMC Dermatol. 2004; 4: 10. 5. Vodegel RM, Jonkman MF, Pas HH, et al. U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases. Br J Dermatol 2004; 151: 112-8. 6. Terra JB, Meijer JM, Jonkman MF, et al. The n- versus u-serration is a learnable criterion to differentiate pemphigoid from epidermolysis bullosa acquisita in direct immunofluorescence serration pattern analysis. Br J Dermatol 2013 Mar 13. doi: 10. 1111/bjd. 12308. 7. Pas HH, Kloosterhuis GJ, Heeres K, et al. Bullous pemphigoid and linear IgA dermatosis sera recognize a similar 120-kDa keratinocyte collagenous glycoprotein with antigenic cross-reactivity to BP180. J Invest Dermatol. 1997; 108: 423-9. Rituximab dose finding in pemphigus Pemphigus is treated by long-term systemic corticosteroids, immuno-suppressive and anti-inflammatory agents, and intravenous immunoglobulins. Pemphigus was an incurable disease until the arrival of rituximab. Rituximab was developed in the late 80s and is the first therapeutic monoclonal antibody. It is a chimeric human-mouse monoclonal antibody, which binds specifically to the transmembrane antigen CD20, expressed on B-lymphocytes from the pre-B-cell stage to the pre-plasma-cell stage leading to their depletion. Rituximab is labelled for CD20+ B-cell non-Hodgkin lymphomas and rheumatoid arthritis. For years rituximab has also been used as an off-label drug for different autoimmune diseases. The first successful treatments with rituximab in patients with blistering diseases were seen in paraneoplastic pemphigus associated with B-cell non-Hodgkin lymphomas.[1] Since then, a remarkable therapeutic effect of rituximab was seen in several open case series with pemphigus.[2,3] The common dose of rituximab was adopted from haematology, namely 375 mg/m2 once a week for four consecutive weeks. We questioned if the dosage of this expensive drug could be lower for off-label use in pemphigus. Since the antibody producing B-cells in pemphigus are not malignant, and a single dose as low as 100 mg/m2 can deplete B-cells, we hypothesized that a lower dose of rituximab might be sufficient for pemphigus. Marcel Jonkman and Barbara Horváth started to give two infusions of 500 mg rituximab in an open series of 15 pemphigus patients, cutting the medicine costs to half compared to the lymphoma schedule, but also added azathioprin. All of our patients (100%) responded with clinical 60 BWEADVSMGFINCORR:Opmaak 1 21-07-2014 17:40 Pagina 60