Naslov
Reminiscence on the Post IID 2008 Satellite International Meeting on Autoimmune Bullous Diseases, Otsu Prince Hotel, Japan, May 17-19, 2008

Autori
Marinović, Branka ; Lipozenčić, Jasna

Izvornik
Acta dermatovenerologica Croatica (1330-027X) 16 (2008), 3; 170-171

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, osvrt, stručni

Ključne riječi
Dsc3 ; pemphigus vulgaris ; pemphigus scoring system (PSS) ABSIS scoring sheet

Sažetak
Hundred and twenty enthusiasts were presented the best they could on topic which become clearer but not totally understood. The biggest organ in our body – the magic skin was lightened on the International Meeting on Autoimmune Bullous Diseases, Otsu, Japan, May 17-19, 2008, Could loss of Dsc3 – a desmosomal cadherin function be a cause of pemphigus vulgaris? Is Dsc 3 hl/pl New born Mutant? Desmoglein 1 (Dsg1) silencing impairs differentiation and morphogenesis of suprabasal keratinocytes. Desmoglein 1 adhesive ectadomain is dispensable for differentiation of keratinocytes. Desmoglein and 2 act as the Yin and Yang of epidermal differentition. Desmoglein 1 regulates GF and acting remodeling pathway via cortactin during keratinocytes differentiation. In Fago Selvagem (FS) is prevalense of IgG AND IgM anti Dsg1 antibodies. In FS cases IgM prevail on IgG and in neonates, but in Japan prevail IgG on IgM. Predictor for FS is IgG4. There are structural determinates of autoantibody pathogenecity in pemphigus and restricted antibody variable region genes. Immunoglobulin E has the role with BP 180 antibody in bullous pemphigoid patients. The dermis contains free BP 180 antigen as a proteolytic fragment. IgE antibody to BP180 fragments bind to mast cells. In EBA is autoantibody response. IgG to type VII collagen inducess subepidermal blisters according passive transfer of IgG from EBA patients. The patients with anti p 200 pemphigoid have positive laminin γ 1 monoclonal antibody in skin and vessels an exclusive p200 antigen. There are in pemphigus acantholysis caused by Dsg and nonDsg factors ; synergeic Dsg and nonDsg action in present and the nonDsg-like acetycholine should be used as model for further research. The role of tight junction (TJ) in epithelial barrier function was elucidated through claudin-based tight junction. An ubiquitin E3 ligase LwX1 is possible involved in the turn over of TJ by internalization and degradation of cloudins in the skin. Single molecule tracking in the living cell membrane: signaling in a variety of membrane domains. The role of p 38 MAPK signaling in pemphigus is necessary. Is p 38 MAPK kinase primary or secondary event of acantholysis? Increased phosphorylation of p 38 MAPK is in pemphigus vulgaris IgG. Inhibitors of p 38 MAPKblock pemphigus vulgaris IgG internalization. Gain or loss of cell-cell adhesion initiates a signal transduction cascade until actin filaments. Pemphigus foliaceus IgG do not directly inhibit Dsg1 transinteraction. Pemphigus vulgaris IgG and AK 23 directly inhibits Dsg1, and PV IgG reduce binding of Dsg3 and DSg 1 to the surface of keratinocytes. Signaling is primary to acantholysis. Catenin p-120 is a possibly new intracellular signaling mediator in pemphigus acantholysis.Desmoglein 3 reactive T-cells: a key player in the autoantibody productions are proved in mouse model with immunotolerance in mouse with rDsg3 in footpad. HLA class II-transgenic mouse is an in vivo model for cellular and humoral immune response to humoral desmoglein 3 in pemphigus vulgaris – HLA-DRO 402-DQ 8. Rituximab (375 mg/m2 1, 8, 15, 22 d) and IVIG (2g/kg/6 months) are powerful new treatment option and bullets for acute recalcitrant pemphigus vulgaris patients. It is suggested that novel rituximab treatment protocol for PV would be better with only twice application of 500 mg or 1 g/kg for 4 days and in 21-22 day. IVIG results as monotherapy with variable dosage: 200 mg, 400 mg revealed no difference. It is suggested that it is 80. day of treatment “ time to escape from the protocol” . The most important was Pemphigus scoring system (PSS): ABSIS scoring sheet ; PDAI and PGA (physician Global Score). The pemphigus vulgaris data should be started preferentially in a data base with Croatian, too. Again, we wish to congratulate on Japanese organization for such scientific success on autoimmune diseases.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA