Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain

Abstract

The prevalence of neoplasms was similar to that seen in inflammatory bowel disease. Multicentric prospective studies including larger numbers of patients are required for a better knowledge and management of this disease.

International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita

“…There has been confusion in the literature as to which forms constitute an ‘inflammatory’ form of EBA . The IBDG members agreed that the BP‐like form is inflammatory but that both MM‐EBA and IgA‐EBA may also be inflammatory.…”

“…The frequency of these subtypes varies in different countries, with the classical type being the most common in European reports and the BP-like form more common in Asia (Table S2). [36][37][38][39] It should be recognized that overlapping clinical presentations may also occur (proposal 09, cases 1-5 in Appendix S2). Overall, because EBA can appear clinically, histologically and immunologically similar to BP, 18,[20][21][22][23][24] when the initial diagnostic consideration of the dermatologist is BP, EBA should also be considered.…”

Mechanisms of Autoantibody-Induced Pathology

“…These include ocular, oral mucosa, esophagus, anal, vaginal, tracheal, and laryngeal lesions, which can lead to blindness, esophageal strictures, hoarseness, impaired phonation, and may led to irreversible respiratory distress (374). Overall, EBA is notoriously difficult to treat, and often long-term use of high doses of corticosteroids in combination with other immunosuppressants is required to achieve clinical remission (375, 376). Thus, there is a high, and so far unmet medical need, for the development of novel treatment strategies for patients with EBA.…”

The use of rituximab in treatment of epidermolysis bullosa acquisita: Three new cases and a review of the literature

“…Table provides an overview of 16 EBA cases treated with rituximab (Cavailhes et al, ; Crichlow et al, ; Iranzo et al, ; Kim et al, ; Lamberts et al, ; McKinley et al, ; Meissner et al, ; Mercader et al, ; Sadler et al, ; Saha et al, ; Schmidt et al, & Wallet‐Faber et al, ). Prior to initiating rituximab therapy, these patients were treated with various agents as presented in Table .…”