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Immunoablative high-dose cyclophosphamide without stem cell rescue in pemphigus foliaceus
Mark A Cohen1, Jonathan J Cohen, Francisco A Kerdel
1Department of Dermatology and Cutaneous Surgery, University of Miami, FL, USA.
International Journal of Dermatology
|July 9, 2002
Summary
High-dose cyclophosphamide without stem cell rescue effectively treats severe pemphigus foliaceus. This immunoablative therapy offers a safe option for recalcitrant autoimmune diseases, showing sustained remission and reduced autoantibody levels.
Area of Science:
- Dermatology
- Immunology
- Oncology
Background:
- Evidence suggests immunoablative high-dose cyclophosphamide without stem cell rescue is effective for refractory autoimmune diseases.
- Conditions include paraneoplastic pemphigus, systemic lupus erythematosus, aplastic anemia, and pemphigus vulgaris.
Observation:
- A 51-year-old patient with severe, treatment-resistant pemphigus foliaceus presented with characteristic skin lesions and high autoantibody titers.
- The patient received a 4-day course of high-dose cyclophosphamide (50 mg/kg/day) and tolerated it well.
Findings:
- Within 3 months, skin lesions healed, and prednisone dosage was reduced.
- Circulating autoantibodies decreased post-treatment, and a mild relapse 10 months later was manageable with lower immunosuppression.
Implications:
- This case supports high-dose cyclophosphamide's efficacy for recalcitrant pemphigus foliaceus.
- The treatment offers a viable alternative for autoimmune diseases unresponsive to conventional therapies.
- Further research into this regimen for autoimmune blistering diseases is warranted.