Paraneoplastic Pemphigus Treatment & Management

Updated: Mar 04, 2016
  • Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Response to treatment paraneoplastic pemphigus is generally poor, especially for mucosal lesions. Initial care is aimed at treating superinfection, if present. Warm compresses, nonadherent wound dressings, and topical antibiotic ointment are helpful. Potent immunosuppressive agents are required to decrease blistering, but they are often ineffective. High-dose corticosteroids are first-line therapy for paraneoplastic pemphigus, [11] followed by steroid-sparing agents such as azathioprine, cyclosporine, and mycophenolate mofetil. In general, the skin lesions of paraneoplastic pemphigus are more responsive to therapy than mucosal lesions. [39]

Other therapeutic options for paraneoplastic pemphigus include plasmapheresis, immunophoresis, intravenous gammaglobulin, [27] and stem cell ablation therapy with high-dose cyclophosphamide [10] without stem cell rescue. Rituximab has been tried in several patients with mixed results. [40, 41] Alemtuzumab, an agent that targets CD52, has also been used with success. [42] Treating the underlying malignancy may control autoantibody production, and intravenous gammaglobulin (IVIG) at the time of surgery may help prevent the development of bronchiolitis obliterans. However, once this develops, it is typically not reversible. [6]

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Surgical Care

For solid neoplasms, curative resection should be attempted when appropriate, but this does not halt disease progression, especially when malignant. If surgery results in decreased autoantibody production, the paraneoplastic pemphigus may improve. IVIG before, during, and after the surgery may block autoantibody released from the tumor. Respiratory symptoms are persistent.

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Consultations

Respiratory therapy may be beneficial when pulmonary involvement from paraneoplastic pemphigus causes respiratory insufficiency. Consultations from a pulmonary medicine specialist, an ophthalmologist, a gastroenterologist, and an otolaryngologist should be obtained when appropriate.

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