Rituximab Treatment for Paraneoplastic Pemphigus Associated with Follicular B-Cell Lymphoma: A Case Report and Review of Treatment Outcome

• Main Authors: Waranaree Winayanuwattikun, M.D., Leena Chularojanamontri, M.D., Papapit Tuchinda, M.D., Penvadee Pattanaprichakul, M.D., Kanokvalai Kulthanan, M.D.
• Format: Article
• Language: English
• Published: Mahidol University 2018-11-01
• Series: Siriraj Medical Journal
• Subjects: Paraneoplastic pemphigus; rituximab; follicular B-cell lymphoma; therapy; bronchiolitis obliterans; Siriraj Med J 2018;70: 523-528
• Online Access: http://www.smj.si.mahidol.ac.th/sirirajmedj/index.php/smj/article/view/2751/2635

Objective: To report a case of paraneoplastic pemphigus (PNP), a rare disease, associated with follicular B-cell lymphoma. Treatment outcomes of PNP associated with follicular B-cell lymphoma following rituximab treatment were also reviewed in this article. Case presentation: We reported a PNP patient presenting with intractable stomatitis and erythema multiforme-like lesions. Skin biopsy from erythema multiforme-liked lesion revealed interface dermatitis and necrotic keratinocytes. Direct immunofluorescence demonstrated immunoglobulin G deposition at intercellular space, as well as complement deposition at dermo-epidermal junction. Indirect immunofluorescence using rat bladder substrate was helpful in confirming the diagnosis of PNP. Further investigations revealed an underlying disease of follicular B-cell lymphoma. He was treated with rituximab and systemic corticosteroids. Improvement of mucocutaneous lesions and lymphoma were noted. Nevertheless, he developed hospital-acquired pneumonia and died from septic shock shortly after receiving conventional chemotherapy. His survival duration was approximately 8 months after diagnosis of PNP. Conclusion: It seemed that rituximab might provide promising benefits for mucocutaneous lesions in PNP patients associated with follicular B-cell lymphoma.