ORAL MANIFESTATIONS OF HYDROA VACCINIFORME–LIKE LYMPHOPROLIFERATIVE DISORDER: CASE SERIES

• Published in: Oral surgery, oral medicine, oral pathology and oral radiology oral medicine, oral pathology and oral radiology, 2022-09, Vol.134 (3), p.e151-e152
• Main Authors: De Cáceres, Cinthia Veronica Bardález López, Rodrigues-Fernandes, Carla Isabelly, De Lima Morais, Thayná Melo, Soares, Ciro Dantas, De Almeida, Oslei Paes, Fonseca, Felipe Paiva, Delgado-Azañero, Wilson
• Format: Article
• Language: English
• ISSN: 2212-4403; 2212-4411
• DOI: 10.1016/j.oooo.2022.01.379

Hydroa vacciniforme–like lymphoproliferative disorder (HVLPD) is a chronic Epstein-Barr virus (EBV)-positive lymphoproliferative disease that may either present as an indolent condition or progress to a systemic T-cell lymphoma. This study presents the clinicopathologic data, microscopic findings, and EBV status of 11 cases of HVLPD with oral involvement. A male predilection (7:4) was observed, with mean patient age of 25.1 years. The lips and buccal mucosa were the most affected sites. Lesions appeared as painful ulcers. The patients also showed facial edema and erythema. Microscopic findings included moderate to severe T-cell infiltrate with angiotropism, angiocentricity, and epidermotropism. Two cases affecting the skin on the lip exhibited a periappendageal lymphocytic infiltrate. Immunohistochemistry reactions confirmed the cytotoxic T-cell (CD8+) phenotype, and in situ hybridization showed the presence of EBV in all cases. The clinical course was aggressive, and all patients died. HVLPD is a rare and aggressive disease that may show oral involvement.