Table 2.
Main drugs used in the treatment of epidermolysis bullosa acquisita.7, 8, 9, 25, 26, 27, 28, 29, 30, 31, 32, 33, 63, 65, 67, 71, 72, 73, 74, 75, 76
| Drug (dose) | Main action mechanisms | Main adverse effects | Laboratory evaluation |
|---|---|---|---|
| Systemic corticosteroid (prednisone 0.5‒1.0 mg/kg/day)a or (prednisone 1.0‒1.5 mg/kg/day)b or (methylprednisolone 1 g/day IV for 3 days)b | Inhibition of cytokines, cytopenias (eosinophils, lymphocytes, monocytes), neutrophilia | Ocular (cataract, glaucoma), metabolic (obesity, diabetes, hypertension, dyslipidemia, osteoporosis, Cushing's syndrome), osteoarticular (femoral head avascular necrosis) | Complete blood count, liver enzymes, renal function, fasting glucose, glycated hemoglobin, total cholesterol and fractions, triglycerides, bone densitometry |
| Dapsonea (50‒100 mg/day) | Anti-neutrophilic action, with reduced chemotaxis of neutrophils | Hemolytic anemia, methemoglobinemia, agranulocytosis, DRESS (drug reaction with eosinophilia and systemic symptoms) | Glucose-6-phosphate dehydrogenase, complete blood count, liver enzymes, lactate dehydrogenase, reticulocytes, total bilirubin and fractions |
| Colchicinea (0.5‒2.0 mg/day) | Anti-neutrophilic action, with inhibition of neutrophil chemotaxis and increase in prostaglandin E2 | Neutropenia, diarrhea, abdominal discomfort | Complete blood count, liver enzymes, kidney function |
| Cyclosporineb (5 mg/kg/day) | Calcineurin phosphatase inhibition, causing depletion of T cells and macrophages, and activation of natural killer cells, T cells, and antigen-presenting cells | Nephrotoxicity, hypertension, hypertrichosis, dyslipidemia, headache | Complete blood count, liver enzymes, kidney function, total cholesterol and fractions, triglycerides |
| Mycophenolate mofetilb (2‒3 g/day) | Inhibition of purine synthesis, causing lymphocyte depletion | Nausea, diarrhea, hepatitis, lymphopenia | Complete blood count, liver enzymes, kidney function, serology for hepatitis B, C, HIV |
| Intravenous immunoglobulin (2 g/kg IV in 3‒5 days) | Depletion of autoantibodies by reducing the half-life of immunoglobulins | Headache, chest pain, fever, dyspnea, myalgia, nausea, vomiting, diarrhea, tachycardia, erythema, anaphylaxis, acute kidney injury, thromboembolism, aseptic meningitis, neutropenia, hemolytic anemia | Complete blood count, renal function, liver enzymes, serum immunoglobulin levels (to rule out IgA deficiency, due to the increased risk of anaphylaxis) |
| Rituximabb (1000 mg D1 and D15 or 375 mg/kg/m2 four weekly doses) | Chimeric anti-CD20 monoclonal antibody that induces B lymphocyte depletion by inducing apoptosis, complement activation and cytotoxicity | Fever, nausea, vomiting, angioedema, bronchospasm, anaphylaxis, infection, hepatitis B reactivation, angina, arrhythmia, heart failure, coronary syndrome | Complete blood count, liver enzymes, kidney function, serology for hepatitis B, C, HIV |
a
Non-severe EBA: absence of mucosal, ocular, laryngeal, esophageal lesions and ≤10% of the affected body surface without functional limitation.
b
Severe EBA: the presence of ocular, laryngeal, esophageal lesions and/or >10% of the affected body surface and/or the functional limitation or non-severe EBA refractory to treatment.