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Hailey-Hailey disease is a blistering genodermatosis that shows acantholytic dyskeratosis throughout the epidermis. The aim of our study was to investigate the involvement of adherens structures and cytofilaments in this particular type of acantholysis. Both lesional and non-lesional skin from 18 patients was studied histologically and ultrastructurally. Additionally, the samples were stained for desmosomes, adherens junctions, keratin filaments, actin filaments, and actin-associated proteins, and finally investigated with an electron and a confocal laser scanning microscope (CLSM), respectively. Acantholytic dyskeratosis was not only confined to lesions, but was also focally detectable in clinically unaffected skin. Despite disruption and internalization of the desmosomes, keratinocytes remained linked together by well-preserved adherens junctions. Staining for actin filaments with fluorochrome-labeled phalloidin showed a remarkable formation of actin stress fibers in these keratinocytes. Thus, incomplete acantholysis, as demonstrable in both lesional and non-lesional skin of Hailey-Hailey patients, may be due to a cohesive function of the adherens junction-actin system succeeding the dissolution of desmosomes. Most remarkably, none of the adnexal epithelia expressed the intrinsic defect of cell adhesion. This finding offers an explanation for the successful treatment of Hailey-Hailey disease by dermabrasion, which after complete removal of the involved epidermis results in reepithelialization from skin appendages.  相似文献   
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A patient presenting with acute severe asthma associated with protracted hypoventilation (paCO2 = 90 mmHg) and impaired consciousness underwent sedation and controlled mechanical ventilation. The peak airway pressure could be reduced by constant-volume mechanical ventilation and analgosedation with midazolam and ketamine. When the gas exchange had stabilized, the first attempt at weaning was made by synchronized intermittent mechanical ventilation (SIMV). After this and two other attempts had failed, the patient was given continuous sufentanil (0.8-1.0 micrograms/kg), as well as additional bolus injections of 2 micrograms/kg and 0.35 micrograms/kg for analgosedation. Controlled mechanical ventilation was replaced by the system of biphasic positive airway pressure (BIPAP). The mechanical ventilatory support was gradually reduced, as was the dose of continuous sufentanil. Extubation was possible at a dose of 0.2 micrograms/kg per h sufentanil and CPAP. From this case, it is not possible to determine whether the BIPAP system or the analgosedation with Sufentanil was the decisive factor in the therapeutic success. It may well have been the combination of both therapeutic measures that--in our opinion--supplemented each other in an ideal way.  相似文献   
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Abstract Cytokines released by mast cells as well as their effects on mast cell functions appear to be of major importance in the pathogenesis of mast cell mediated skin diseases. In addition, the identification of some key mediators which were found to play a crucial in vivo rôle in certain disease states may allow the development of new therapeutie strategies using cytokines or cytokine antagonists for the treatment of inflammatory skin diseases. So far, encouraging results have been obtained when diseases with mast cell involvement such as atopic eczema or mastocytosis have been treated with different IFNs. Future trials using IFNs and other newly detected cytokines or their antagonists are required to establish effective therapy regimens.  相似文献   
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