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

Abstract

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. Am.

Rituximab in pemphigus

“…[13] Subsequently many authors have used rituximab in the treatment of various immunobullous diseases including PV [14] and PF. [15] Indications Rituximab is indicated in pemphigus patients who fail to respond adequately to conventional treatment modality, or in whom systemic corticosteroids and/ or other immunosuppressants are contraindicated or cause severe adverse effects requiring its discontinuation.…”

Diagnosis and management of oral mucosal lesions in older people: a review

“…54 The bullae of pemphigoid are tense and raised and not as easily ruptured because the blister roof is composed of a full thickness epithelium. Oral bullae tend to occur first, which rupture to produce ragged oral ulcers, followed by cutaneous lesions.…”

Therapy of autoimmune bullous diseases

“…One patient with PNP and chronic lymphocytic leukemia had marked improvement with high dose cyclophosphamide therapy without stem cell rescue (Nousari, Brodsky, et al 1999). Rituximab was effective in a patient with PNP associated with CD20+ follicular lymphoma (Borradori et al 2001) and a case of PNP associated with follicular non-Hodgkin’s lymphoma (Heizmann et al 2001). The dose is 375 mg/m 2 intravenously every week.…”