Lymphomatoid Papulosis “Type E” Affecting the Palate: A Detailed Case Report and Review of Literature

Abstract

Abstract: Lymphomatoid papulosis (LyP) belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, characterized by chronic, recurrent, self-healing papules, small nodules, or ulcers. The clinicopathological features of LyP can mimic overt lymphomas. To date, about 27 intraoral LyP cases have been reported. Of them, only 2 cases were diagnosed as angioinvasive LyP (type E). Herein, we report a 24-year-old Brazilian man who presented a large ulcerated lesion on the hard palate with rapid evolution. Remarkably, there was no involvement of the skin or other mucous membranes. Microscopy revealed a lymphoid infiltrate constituted by medium-sized to large atypical cells, with angiocentric and angiodestructive features. The atypical cells showed immunopositivity for CD3, CD8, CD30, CD56, granzyme B, perforin, and focally for MUM1/IRF4. Ki-67 highlighted almost all atypical lymphoid cells, whereas EBER1/2 was negative. After 2 months of follow-up, the lesion healed completely. Although rare, LyP type E should be included in the differential diagnosis of oral ulcers.