Generalized Bullous Pemphigoid Unresponsive Despite Intensive Combined Topical and Systemic Corticosteroid Therapy
A subset of patients with generalized bullous pemphigoid fail to reach an adequate level of disease control even after several weeks of intensive treatment combining topical and systemic corticosteroids. This refractory presentation requires a structured reassessment of therapeutic options.
Clinical scenario: Generalized bullous pemphigoid that remains below the controllable level (unresponsive) despite several weeks of intensive combined topical and systemic corticosteroid therapy.
Therapeutic approach (partial): For patients who do not respond to intensive corticosteroid therapy, several non-validated alternative therapies are considered — including agents targeting immune pathways. The complete protocol specifies which interventions apply and how to select among them.
References
- In the cases of those few patients with generalized disease who remain below the controllable level (unresponsive) despite several weeks of intensive therapy with combined topical and systemic CS, there are the following therapeutic options:
- B-cell depletion therapy with anti-CD20 mAb (rituximab) (4.04 ± 1.74);
- For B-cell depletion therapy with anti-CD20 monoclonal antibody (mAb) using rituximab, it is recommended to apply either the rheumatoid arthritis protocol (two 1 g intravenous infusions, 2 weeks apart) or the lymphoma protocol (375 mg/m2 intravenous infusion, once weekly for 4 weeks) (4.66 ± 0.70).
- Omalizumab (4.31 ± 0.89);
- Dupilumab (4.30 ± 0.86);
- Intravenous immunoglobulins (4.57 ± 0.59);
- Immunoadsorption (4.23 ± 1.13).
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