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"A Fluid Filled" Presentation of Lymphoma
"A Fluid Filled" Presentation of Lymphoma
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A 69-year-old man with no significant prior medical history developed a 6-week history of progressively worsening painful mucocutaneous lesions involving the oral cavity, face, scalp, trunk, extremities, and groin. Initially treated as a fungal infection, his condition rapidly worsened with severe oral erosions, bullous skin lesions, constipation, 30-pound weight loss, lethargy, and malaise. Imaging of the abdomen and pelvis showed an 18-cm retroperitoneal mass, retroperitoneal lymphadenopathy, left hydronephrosis, and a left pleural effusion.<br /><br />Biopsy of a skin lesion showed suprabasilar acantholysis, and pleural fluid cytology revealed high-grade B-cell lymphoma. Biopsy of the retroperitoneal mass confirmed follicular lymphoma. Based on the clinical picture and pathology, the patient was diagnosed with paraneoplastic pemphigus (PNP) associated with lymphoma. He was treated with high-dose intravenous corticosteroids followed by urgent R-CHOP chemotherapy, which stabilized his condition.<br /><br />PNP is a rare and often fatal mucocutaneous autoimmune syndrome most commonly linked to an underlying untreated lymphoproliferative disorder. It accounts for 3–5% of pemphigus cases and carries a high mortality rate, largely due to severe mucosal disease and potential respiratory involvement such as bronchiolitis obliterans. The hallmark feature is severe erosive stomatitis, often accompanied by polymorphic skin findings including erosions, bullae, erythema, and scaly plaques. Diagnosis relies on clinical presentation, histopathology, immunofluorescence, and/or immunoprecipitation.<br /><br />This case highlights the importance of recognizing progressive painful mucocutaneous disease as a possible manifestation of occult malignancy, emphasizing the need for early diagnosis and prompt treatment of the underlying cancer.
Asset Subtitle
Kevin C. Eaton
Meta Tag
Author List
Kevin C. Eaton, Neha Verma, Phillip Jules
Category
Clinical Vignettes
Concept
Paraneoplastic Pemphigus
Concept
Erosive Stomatitis
Concept
Lymphoproliferative Malignancy
Concept
Lymphoma
Concept
Malignancy
Distinguished
Non-Finalist
Presenter Organization
Moffitt Cancer Center
Presenting Author
Kevin C. Eaton
Track
Adult
Keywords
paraneoplastic pemphigus
follicular lymphoma
high-grade B-cell lymphoma
mucocutaneous lesions
oral erosions
suprabasilar acantholysis
retroperitoneal mass
R-CHOP chemotherapy
bullous skin lesions
bronchiolitis obliterans
Paraneoplastic Pemphigus
Erosive Stomatitis
Lymphoproliferative Malignancy
Lymphoma
Malignancy
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