BACKGROUND: Paraneoplastic pemphigus (PNP) in the oral cavity is a rare variant of blistering pemphigus disease closely associated with mostly malignant tumors. The diagnosis may even precede an underlying malignancy enabling early detection. Here, we describe a previously unreported case of PNP associated with HPV-related tonsillar squamous cell carcinoma. METHODS AND RESULTS: A 50-year-old woman was referred to a dentist because of painful oral lesions resembling aphthae major and minor. Later, blisters appeared and an incisional biopsy was performed. Histological examination revealed an unusual coexistence of subepithelial and intraepithelial blisters raising suspicion of paraneoplastic pemphigus. The patient underwent 18F-FDG PET/MRI, showing a metabolically active process in the left palatal tonsil. Diagnostic biopsy revealed HPV type 16 associated tonsillar squamous cell carcinoma. A left tonsillectomy with elective left-sided neck dissection was performed. The postoperative period was complicated by bilateral fluidothorax. Two weeks after radical tumor removal, the mucosal and skin lesions of PNP disappeared. The patient currently shows no evidence of recurrence either of malignancy or PNP eight months after the surgery. CONCLUSION: PNP is a rare autoimmune blistering disease characterized by polymorphous cutaneous and mucosal lesions associated with internal neoplasms including HPV associated squamous cell carcinoma of a tonsil. In order to identify an occult malignancy, a whole-body PET/CT or PET/MRI scan is recommended. Rarely, accurate patient management may depend on the dentist being familiar with this entity and on interdisciplinary cooperation involving dermatologist, radiologist, pathologist, and pneumologist. A strict patient follow-up is indicated.

Biopticka Laboratory Mikulasske namesti 628 326 00 Pilsen Czech Republic

Department of Dentistry University Hospital and Faculty of Medicine in Pilsen Charles University Alej Svobody 80 304 60 Pilsen Czech Republic

Department of Imaging Methods University Hospital and Faculty of Medicine in Pilsen Charles University Alej Svobody 80 304 60 Pilsen Czech Republic

Department of Otorhinolaryngology University Hospital and Faculty of Medicine in Pilsen Charles University Alej Svobody 80 304 60 Pilsen Czech Republic

Sikl's Department of Pathology Faculty of Medicine in Pilsen Charles University Ed Benese 13 305 99 Pilsen Czech Republic

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