blistering. Diagnosis of EBA is based on history, full skin examination, and skin biopsies. There is limited data on treatment options for EBA and optimal approach to treatment has not been established. Suggested initial treatment is with colchicine or dapsone (Grade 2C). If treatment is not effective, these agents may be used simultaneously. EBA that is refractory to above requires more aggressive therapy. Agents that may have efficacy for refractory EBA include immunosuppressants, intravenous immunoglobulin, and rituximab. Diagnosis is Yes By which specialty Dermatologist Treatment of will be limited to required dermatologists and clinical immunologists as Clinical it is a rare condition, usually treated by Immunologist these specialists. Diagnosis must No By which specialty be verified Exclusion Criteria Persistent severe EBA refractory to conventional Qualifying The definition of persistent and severe immunosuppressive therapy Criteria disease for eligibility for Ig therapy has been limited to those patients with Persistent severe EBA disease confirmed by biopsy and ophthalmological and /or mucosal disease. Immunofluorescence including ophthalmological and/or mucosal sites AND Persistent disease despite standard treatment with The use of Ig therapy is as a fifth line agent colchicine and dapsone and at least two other after failure to respond to alternative immunosuppressant agents therapies unless contraindicated or an OR unacceptable level of side effects or toxicity
blistering. Diagnosis of EBA is based on history, full skin examination, and skin biopsies. There is limited data on treatment options for EBA and optimal approach to treatment has not been established. Suggested initial treatment is with colchicine or dapsone (Grade 2C). If treatment is not effective, these agents may be used simultaneously. EBA that is refractory to above requires more aggressive therapy. Agents that may have efficacy for refractory EBA include immunosuppressants, intravenous immunoglobulin, and rituximab. Diagnosis is required Yes By which specialty Dermatologist Clinical Immunologist Treatment of will be limited to dermatologists and clinical immunologists as it is a rare condition, usually treated by these specialists. Diagnosis must be verified No By which specialty Exclusion Criteria Qualifying Criteria Persistent severe EBA refractory to conventional immunosuppressive therapy Persistent severe EBA disease confirmed by biopsy and Immunofluorescence including ophthalmological and/or mucosal sites AND Persistent disease despite standard treatment with colchicine and dapsone and at least two other immunosuppressant agents OR The definition of persistent and severe disease for eligibility for Ig therapy has been limited to those patients with ophthalmological and /or mucosal disease. The use of Ig therapy is as a fifth line agent after failure to respond to alternative therapies unless contraindicated or an unacceptable level of side effects or toxicity
Epidermolysis-bullosa-acquisita2017.docx