Case report demonstrates targeted therapy improved outcomes in a patient with paraneoplastic pemphigus, indicating a role for cellular immunity in treatment.
Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disorder often resistant to conventional immunosuppressive therapies. We report a case of refractory PNP associated with follicular dendritic cell sarcoma, in which functional T cell analysis revealed a dominant Th1/Tc1 immune response and elevated serum TNF, guiding successful treatment with the TNF inhibitor etanercept. This case underscores the potential of molecular-guided therapy targeting cellular immunity in managing refractory PNP.
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Shih et al. (2025) studied this question.
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