Immunoapheresis in Paraneoplastic Pemphigus

Abstract

Background: Paraneoplastic pemphigus was first described in 1990 in 5 patients with extensive mucocutaneous erosions, a distinct set of autoantibodies, and underlying neoplasia. Since then, patients described have been middle-aged, have suffered from prognostically unfavorable malignant neoplasms, and have responded poorly to immunosuppressive agents.Observation: A 16-year-old boy was examined with extensive oral erosions, halitosis that interfered with his quality of life, and rapid weight loss. The suspected clinical diagnosis of paraneoplastic pemphigus was confirmed by histopathological, immunofluorescence, and biochemical (eg, immunoblotting and immunoprecipitation) findings as well as by the demonstration of an inflammatory myofibroblastic tumor of the left retroclavicular region.Despite administration of corticosteroids, followed by excision of the neoplasm, clinical symptoms improved only slightly, and autoantibody titers decreased only marginally. We therefore initiated an immunoapheresis regimen with the use of sheep anti-human-IgG beadformed agarose gel (Sepharose; Pharmacia Biotech Comp, Vienna, Austria), which led to the disappearance of circulating autoantibodies and the patient's recovery. Conclusion:Immunoapheresis may represent a novel therapeutic option for patients with paraneoplastic pemphigus who show little improvement after curative treatment of their neoplasms.

Paraneoplastic Pemphigus: A Case of Long-Term Survival Associated with Systemic Lupus erythematosus and Polymyositis

“…The only exception would be patients with benign neoplasms like thymomas or the hyaline-vascular type of Castleman disease that can often be completely excised [2]. A great variety of immunosuppressive therapies have been tried in PNP, including oral and intravenous prednisone, oral and intravenous cyclophosphamide, azathioprine, dapsone, gold, cyclosporin, plasmapheresis, photopheresis and immunoapheresis [2, 16, 17, 18, 19]. Until now the only therapeutic regimen holding some promising results has been the combination of prednisone with cyclosporin or cyclophosphamide [2, 17].…”

Plasmapheresis/immunoadsorption in patients with pemphigus

“…Plazmaferez ve immünadsorpsiyon, Goodpasture sendromu, myastenia gravis ve romatoid artrit gibi antikor aracılı hastalıkların tedavisinde kullanılmaktadır. Plazmaferez pemfigus vulgariste ilk kez 1978 yılında, immünadsorbsiyon ise PNP 1998 yılında kullanılmıştır 8 ; PF, büllöz pemfigoid 9 , pemfigoid gestasyones 10,11 , anti p-200 pemfigoidi 12 ve akiz epidermolizis bülloza 13 tedavisinde de kullanıldığına ilişkin yayınlar mevcuttur. Literatürde PV'de plazmaferez kullanımına dair mevcut olan tek kontrollü çalışma Guillaume ve ark.…”

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“…The Nonhematologic neoplasms associated with PNP include follicular dendritic cell sarcoma [5,42,46,47,72,84,85], inflammatory myofibroblastic tumor [86], leiomyosarcoma [87], liposarcoma [88], malignant nerve sheath tumor [89], inflammatory fibrosarcoma [90], reticulum cell sarcoma [91], and malignant melanoma [92], squamous cell carcinoma of the vagina [93], and adenocarcinoma of the colon [55], prostate [94,95], pancreas [88,93], breast [36], and lung [96,97] (Table 1). Follicular Dendritic Cell Sarcoma (FDCS) is a rare tumor associated with paraneoplastic pemphigus.…”