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High-dose immune suppression and autologous hematopoietic stem cell transplantation in refractory pemphigus foliaceus

Bone Marrow Transplantation volume 34, pages 1097–1098 (2004)Cite this article

Although autologous hematopoietic stem cell transplantation (HSCT) has been used as treatment for numerous autoimmune diseases,1, 2, 3 bullous autoimmune disorders have not, to our knowledge, been treated by HSCT. Pemphigus foliaceus (PF) is an autoimmune disease with antibody titers against desmoglein-1 (Dsg-1)4 that correlate with the extent and activity of disease. Treatment refractory cases of PF occur and the cause of death is generally infection.

We report a patient treated under an Institutional Review Board and the United States Food and Drug Administration approved protocol for refractory bullous skin diseases that include PF, pemphigus vulgaris (PV), and cicatricial pemphigoid (CP). Patients on this protocol must have failed to respond to prednisone 0.5 mg/kg/day for more than 3 months and at least two other immunosuppressive agents that include cyclophosphamide (CY), mycophenolate mofetil (MMF), gold, cyclosporine, methotrexate (MTX), or plasmapheresis. Failure is defined as the involvement of more than 10% of body surface area, mucosal lesions or recurrent infections requiring hospitalizations.

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Authors and Affiliations

  1. Division of Immunotherapy, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Y Oyama, L Statkute, L Verda & R K Burt

  2. Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    E R Parker, J Brieva & J Guitart

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  1. Y Oyama
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  2. E R Parker
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  7. R K Burt
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Oyama, Y., Parker, E., Brieva, J. et al. High-dose immune suppression and autologous hematopoietic stem cell transplantation in refractory pemphigus foliaceus. Bone Marrow Transplant 34, 1097–1098 (2004). https://doi.org/10.1038/sj.bmt.1704679

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