ABSTRACT
Introduction and importance: Paraneoplastic pemphigus (PNP) is an uncommon autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphous skin eruptions, and the presence of underlying neoplasms. Unique histopathological features include suprabasal acantholysis and clefts with scattered necrotic keratinocytes. Case presentation: A 27-year-old female patient presented with a 3-month history of a painless swelling, approximately the size of a pea, on the left lateral aspect of her neck and axillary area. This swelling progressively increased in size and number. Additionally, she had reddish, itchy, raised skin lesions over her elbows bilaterally, which gradually spread to involve most of her body, including her lips, tongue, and buccal mucosa. These skin lesions were associated with difficulty swallowing both liquid and solid foods. A diagnostic test, including a biopsy, confirmed the diagnosis of PNP. Subsequently, the patient was managed with chemotherapy and other supportive measures, leading to improvement and eventual discharge. Clinical discussion: PNP is a rare blistering disorder associated with neoplasms, often presenting diagnostic and treatment challenges. Patients with PNP may develop a diverse range of lesions. It is crucial to promptly recognize and manage the underlying malignancy for improved patient outcomes. Conclusion: This case highlights the rare association between T-cell lymphoma and PNP. Clinicians should also remain vigilant for the possibility of PNP in lymphomas that are not of B-cell lineage.
