Bullous pemphigoid: physiopathology, clinical features and management.
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.
Di Zenzo
Giovanni
G
Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Via Monti di Creta 104, 00167 Rome, Italy. g.dizenzo@idi.it
Marazza
Gionata
G
Borradori
Luca
L
D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D002352:Carrier Proteins / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000276:immunology; D000071821:Dystonin; D022002:Hemidesmosomes / Q000276:immunology; D006801:Humans; D007159:Immunologic Tests; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009419:Nerve Tissue Proteins / Q000276:immunology; D024041:Non-Fibrillar Collagens / Q000276:immunology; D010391:Pemphigoid, Bullous / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D016032:Randomized Controlled Trials as Topic; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D007166:Immunosuppressive Agents; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human