Paraneoplastic pemphigus with airway involvement

Area of Science:

• Pulmonology
• Dermatology
• Oncology

Background:

• Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease often associated with hematologic malignancies.
• Airway involvement in PNP is uncommon but can lead to severe respiratory compromise.

Observation:

• A patient presented with acute hypoxemic respiratory failure attributed to bronchiolitis obliterans.
• The patient had a confirmed diagnosis of paraneoplastic pemphigus.

Findings:

• High-dose systemic corticosteroids and rituximab were administered.
• Niacinamide and doxycycline were included as part of the therapeutic regimen.
• Significant improvement in respiratory function was observed following treatment.

Implications:

• This case suggests a potential therapeutic benefit of niacinamide and doxycycline in managing airway involvement in PNP.
• The findings expand treatment options for paraneoplastic pemphigus with pulmonary manifestations.
• Further research is warranted to confirm the efficacy of this combination therapy in larger cohorts.