Therapy of paraneoplastic pemphigus with Rituximab: A case report

Area of Science:

• Autoimmune diseases
• Oncology
• Dermatology

Background:

• Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease often associated with hematologic malignancies or thymoma.
• Treatment options for PNP are limited and often involve immunosuppressants and management of the underlying malignancy.
• Thymoma is a common neoplastic association with PNP, necessitating effective therapeutic strategies.

Observation:

• A 39-year-old female patient diagnosed with paraneoplastic pemphigus (PNP) associated with thymoma presented with severe oral ulcerations and mucocutaneous lesions.
• The patient was treated with a combination regimen including rituximab, corticosteroids, and methotrexate.
• Clinical response was monitored over an 8-week period.

Findings:

• Following 8 weeks of rituximab-containing therapy, the patient experienced complete resolution of oral ulcerations.
• Significant improvement was observed in cutaneous lesions affecting the trunk and limbs.
• The combination therapy demonstrated efficacy in managing the symptoms of PNP.

Implications:

• Rituximab, an anti-CD20 monoclonal antibody, shows potential as an effective therapeutic agent for paraneoplastic pemphigus.
• This case highlights the benefit of integrating rituximab into the treatment protocol for PNP associated with thymoma.
• Further clinical studies are essential to validate the efficacy and safety of rituximab in larger cohorts of PNP patients.