Di Zenzo Giovanni, Marazza Gionata, Borradori Luca
Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Via Monti di Creta 104, 00167 Rome, Italy.
Adv Dermatol. 2007;23:257-88. doi: 10.1016/j.yadr.2007.07.013.
There has been a considerable progress in the understanding of the physiopathology of BP during the past 2 decades. The insights into the humoral and cellular immune response against BP180 and BP230 have increased significantly. Nevertheless, the factors underlying the initiation of the disease leading to a disruption of self-tolerance remain unclear. Clinically, the disease shows protean presentations, and diagnostic delay is common. A practical, relevant, and unresolved question is how to identify patients suffering from BP at an early stage of the disease, when direct immunofluorescence microscopy findings still may be negative. The characterization of markers allowing the differentiation of BP from other pruritic eruptions occurring in the elderly population would be extremely helpful in daily practice. Finally, despite the knowledge that potent topical steroids are efficient in controlling the disease, management of BP sometimes remains difficult and requires systemic therapies. It is hoped that a better knowledge of the regulation of the autoimmune response in BP also will facilitate the design of novel immunomodulatory therapeutic approaches devoid of the severe side effects of current immunosuppressive treatments.
在过去20年里，人们对大疱性类天疱疮（BP）的生理病理学的理解有了长足的进展。对针对BP180和BP230的体液和细胞免疫反应的认识显著增加。然而，导致自身耐受破坏的疾病起始的潜在因素仍不清楚。临床上，该疾病表现多样，诊断延迟很常见。一个实际、相关且未解决的问题是，在疾病早期，当直接免疫荧光显微镜检查结果可能仍为阴性时，如何识别患有BP的患者。能够区分BP与老年人群中出现的其他瘙痒性皮疹的标志物的特征，在日常实践中将非常有帮助。最后，尽管已知强效外用类固醇对控制该病有效，但BP的管理有时仍然困难，需要进行全身治疗。希望对BP自身免疫反应调节的更好了解也将有助于设计新的免疫调节治疗方法，避免当前免疫抑制治疗的严重副作用。
