nonresponsive to conventional treatments, were started on 2 g/kg/cycle of IVIg for a mean of 23 cycles over 39 months. All 10 demonstrated clinical response and were able to completely withdraw their previous therapy; no recurrence was observed during a mean follow-up period of 54 months after cessation of treatment. Persistent severe EBA refractory to conventional Intravenous Two indications have been developed, the immunosuppressive therapy. Indications immunoglobulin (IVIg) first to provide three months of initial Ig should be considered therapy in eligible severe refractory patients. for severe cases All patients will then have a trial off Ig Worsening of EBA disease in responding patients with ongoing refractory to therapy and if worsening of EBA disease flare after ceasing Ig therapy conventional should occur, patients may be eligible for immunosuppressive maintenance therapy provided a response to therapy. initial therapy can be demonstrated. Epidermolysis bullosa acquisita is a rare, potentially severe Description and This section has been developed following disease which has no cure. This sub-epidermal Diagnostic assessment of the literature and consultation blistering disorder of the skin and mucous membranes primarily Criteria with the Australasian College of affects adults but has been described in a few children. Although Dermatologists. the majority of patients may have mild disease with blistering over trauma prone areas, there have been cases of severe widespread disease that remains refractory to conventional therapies. The end stage results of severe disease include ocular disease with conjunctival scarring and blindness, mucosal disease with oesophageal strictures and scarring fibrosis of the skin with alopecia, nail loss and mitten like deformities of the hands. EBA is an autoimmune disease characterized by the production of antibodies against type VII collagen resulting in immune-mediated disruption of the anchoring fibrils that connect the basement membrane to dermal structures and the clinical development of
nonresponsive to conventional treatments, were started on 2 g/kg/cycle of IVIg for a mean of 23 cycles over 39 months. All 10 demonstrated clinical response and were able to completely withdraw their previous therapy; no recurrence was observed during a mean follow-up period of 54 months after cessation of treatment. Indications Intravenous immunoglobulin (IVIg) should be considered for severe cases refractory to conventional immunosuppressive therapy. Persistent severe EBA refractory to conventional immunosuppressive therapy. Worsening of EBA disease in responding patients with ongoing flare after ceasing Ig therapy Two indications have been developed, the first to provide three months of initial Ig therapy in eligible severe refractory patients. All patients will then have a trial off Ig therapy and if worsening of EBA disease should occur, patients may be eligible for maintenance therapy provided a response to initial therapy can be demonstrated. Description and Diagnostic Criteria Epidermolysis bullosa acquisita is a rare, potentially severe disease which has no cure. This sub-epidermal blistering disorder of the skin and mucous membranes primarily affects adults but has been described in a few children. Although the majority of patients may have mild disease with blistering over trauma prone areas, there have been cases of severe widespread disease that remains refractory to conventional therapies. The end stage results of severe disease include ocular disease with conjunctival scarring and blindness, mucosal disease with oesophageal strictures and scarring fibrosis of the skin with alopecia, nail loss and mitten like deformities of the hands. EBA is an autoimmune disease characterized by the production of antibodies against type VII collagen resulting in immune-mediated disruption of the anchoring fibrils that connect the basement membrane to dermal structures and the clinical development of This section has been developed following assessment of the literature and consultation with the Australasian College of Dermatologists.
Epidermolysis-bullosa-acquisita2017.docx