Intravascular large B-cell lymphoma (IVLBCL) presenting with exclusive cutaneous involvement, no B-symptoms and negative PET-CT: A Case Study

Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of extranodal non-Hodgkin lymphoma (NHL) that typically involves the central nervous system (CNS) and skin in its classic (Western) variant and frequently presents with systemic B-symptoms. We present a rare case of IVLBCL in a 59-year-old female with cutaneous-only involvement, no B-symptoms, and negative PET-CT. The patient's initial presentation mimicked inflammatory conditions, contributing to diagnostic delay. Deep skin biopsy was crucial and confirmed the diagnosis of IVLBCL. PET-CT was non-revealing, showing limitations in cutaneous-dominant disease. The patient underwent R-CHOP treatment along with CNS prophylaxis, achieving complete remission with no relapse at follow-up. This case emphasizes the importance of high suspicion and early biopsy in atypical cutaneous presentations and the need for personalized strategies.