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Surgical management with CO2 laser for pseudosyndactyly in recessive dystrophic epidermolysis bullosa

pathogenic in the four cases. The conserved Glu residue of the c2-chain is necessary for integrin binding by laminin332. These four cases may contain autoantibodies directed to this region. Our present results also have some limitations in interpretation because the 3-D structure of pathogenic epitopes from laminin trimers in vivo is lost in the reduced condition in the immunoblot. Keratinocyte necrosis was observed in the roof of the blister in our case. These necrotic keratinocytes could be seen in paraneoplastic pemphigus, herpes gestationis and BP developed after graft-versus-host disease. We previously reported a patient who had developed bullous pemphigoid after graft-versus-host disease and showed apoptosis in the epidermis, in which caspase 3 was cleaved in some keratinocytes facing the blister. Future studies should be performed to detect cleavage of caspases 3 and 8 in keratinocytes to analyze the role of caspase activation on blister formation in MMP.