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ePaper created 2014-09-15, 12:31:02 | version 1.32.01
relatively high fluorescence background in the dermis evoked by aspecific bound circulating IgG. The value of saline as a transport medium was serendipitously found by Jonkman in the 90s when he used saline as a replacement for unavailable liquid nitrogen to transport IF biopsies during a consultation at the weekend. Vodegel systematically studied IF biopsy transport and concluded in 2004 that saline for 24 hours at room temperature (without freezing) improves the desired IF signal and therefore the diagnostic yield in routine direct IF for autoimmune skin diseases.[4] The superior results by saline incubation were explained by washing out aspecific IgG from the skin sample. The use of saline as a transport medium is easy, inexpensive and convenient, but transport time is limited to 36 h. In the early 90s, after seeing hundreds of biopsies of pemphigoid patients it appeared to De Jong that the linear pattern of immunodeposits along the epidermal basement membrane zone is serrated like the edges of leaves. As a biologist he was trained to differentiate leaves by their serration pattern. Vodegel systematically investigated the serration pattern and reported in 2004 that u-form serration is only seen in epidermolysis bullosa acquisita or bullous systemic lupus erythematosus, and that the n-form serration was only found in the other pemphigoids.[5] The u-serration pattern represents immunoglobulin depositions in upstanding arms (“grass”) of the sublamina densa zone between the rootlets of basal keratinocytes. In 2013 J. Terra and co-workers created an image-based online test and instruction video available free of charge (www.nversusu.umcg.nl). The accessibility of direct IF serration pattern analysis was demonstrated among experts, dermatologists and residents using this online IF instruction and testing system.[6] Meanwhile immunoserological diagnosis by immublot was developed by Hendri Pas. He demonstrated that a 120 kDa molecule was bound by both bullous pemphigoid IgG autoantibodies and linear IgA dermatosis IgA autoantibodies, and showed that it was identical to the ectodomain of BP180, which is the major antigen of various forms of pemphigoid.[7] The importance of this discovery was that it demonstrated that bullous pemphigoid and the lamina lucida form of linear IgA dermatosis were diseases that targeted the same antigen. Pas also suggested that the 120 kDa form of the BP180 molecule could be the proteolytically cleaved-off ectodomain of BP180, and that in this process neoepitopes might be formed which explained the observation that some patient sera only bound to the 120 kDa molecule but not to BP180.[7] 59 Bullous pemphigoid. BWEADVSMGFINCORR:Opmaak 1 21-07-2014 17:40 Pagina 59