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Fatal paraneoplastic pemphigus associated with a mediastinal tumor
Katharina Pfistershammer1, Gerald Minimair, Dagmar Födinger
1Department of Dermatology, Division of Immunodermatology and Infectious Skin Diseases, Medical University of Vienna, Austria.
Summary
Paraneoplastic pemphigus (PNP), a rare autoimmune blistering disease, is linked to cancer. This case highlights a fatal outcome despite tumor removal and immunosuppression in a patient with dendritic cell sarcoma.
Area of Science:
- Dermatology
- Oncology
- Immunology
Background:
- Paraneoplastic pemphigus (PNP) is a rare, severe autoimmune blistering disease associated with an underlying neoplasm.
- It presents with mucocutaneous lesions and is often refractory to treatment.
Observation:
- A 34-year-old woman developed severe stomatitis and a widespread lichenoid rash.
- Clinical, immunofluorescence, histopathology, and biochemical analyses confirmed a diagnosis of PNP.
- CT imaging revealed a mediastinal mass, later diagnosed as dendritic cell sarcoma.
Findings:
- Despite surgical resection of the tumor and initiation of immunosuppressive therapy, the patient's PNP rapidly progressed.
- The patient ultimately succumbed to septic multiorgan failure.
Implications:
- This case underscores the critical association between PNP and specific malignancies, such as dendritic cell sarcoma.
- It highlights the often poor prognosis and challenges in managing PNP, even with aggressive treatment.
- Early diagnosis and prompt management of the associated neoplasm are crucial, though not always curative.
