Successful treatment of paraneoplastic pemphigus in follicular NHL with rituximab: report of a case and review of treatment for paraneoplastic pemphigus in NHL and CLL.

Paraneoplastic pemphigus is a severe mucocutaneous disease associated in most cases with B-cell lymphoproliferative disorders. Independent of the course of the underlying malignancy, this autoimmune skin disease is resistant to any treatment in most cases and may lead to death by infectious complications. We observed a patient with recovery of paraneoplastic pemphigus associated with follicular non-Hodgkin's-lymphoma after therapy with rituximab. In addition, we reviewed the literature and analyzed the few cases with comparable outcome with other treatment modalities.