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 bead-formed 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.
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- Autoimmune blistering disorders (AIBD) are conditions where the body produces auto-antibodies against adhesion proteins in the skin, detectable through direct immunofluorescence (DIF) or blood tests like ELISA.
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