LY19 Diagnostic challenges in intraoral lymphomatoid papulosis: a case series

Abstract

Intraoral lymphomatoid papulosis (IoLyP) is an exceptionally rare CD30+ T-cell lymphoproliferative disorder, with fewer than 30 cases reported in the literature. It is characterized by recurrent papulonecrotic or nodular erosive lesions of the oral mucosa that resolve spontaneously, often leaving residual scarring. Lesions typically follow a predictable course, progressing from papule to nodule before resolution. IoLyP may occur in isolation or in association with cutaneous lymphomatoid papulosis and is regarded as the mucosal counterpart of cutaneous disease, sharing the same histological subtypes (A–E). However, its rarity, histological heterogeneity and intermittent clinical presentation frequently result in diagnostic delay, histological uncertainty and complex referral pathways. We report three new cases of IoLyP presenting over a 12-month period, illustrating these challenges. Patient 1 was an 82-year-old man referred to oral and maxillofacial surgery with a 3-week history of dorsal tongue ulceration that resolved prior to review but recurred within 3 months at a different site. Biopsy demonstrated atypical CD30+, CD7− lymphoid cells expressing granzyme B and GATA3. An initial diagnosis of mature T-cell lymphoma was revised to IoLyP following haematology multidisciplinary team review and normal staging imaging, prompting dermatology referral. Patient 2, a 48-year-old woman, presented with a 2-week history of a well-demarcated oral erosion. Biopsy revealed large CD2+ CD4+ CD5+ lymphoid cells with variable CD30 expression, CD7 loss and clonal T-cell receptor rearrangement. Although she was initially referred for haematological assessment and staging, subsequent dermatology review identified previously unrecognized cutaneous lymphomatoid papulosis, allowing systemic immunosuppression to be deferred. Patient 3, a 53-year-old woman, presented to oral and maxillofacial surgery with a necrotic floor-of-mouth lesion that resolved spontaneously within 4 weeks. Histology demonstrated a clonal CD30+ T-cell infiltrate consistent with IoLyP. Multidisciplinary team review confirmed the diagnosis, and dermatology follow-up established a management plan for potential recurrence, with no active treatment required at present.