PT  - JOURNAL ARTICLE
AU  - Liska, Jan
AU  - Liskova, Veronika
AU  - Trcka, Ondrej
AU  - Ondic, Ondrej
AU  - Hauer, Lukas
AU  - Baxa, Jan
AU  - Kacerovska, Denisa
TI  - Oral presentation of paraneoplastic pemphigus as the first sign of tonsillar HPV associated squamous cell carcinoma. A case report.
DP  - 2022 Dec 7
TA  - Biomedical papers
PG  - 447--450
VI  - 166
IP  - 4
AID - 10.5507/bp.2021.039
IS  - 12138118
AB  - 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.