Unilateral lower extremity lymphedema as a first symptom of primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT)

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), is a rare and aggressive variant of extranodal lymphoma. We report the case of a 64-year-old woman with unilateral lymphedema of the lower limb as the first and only symptom of PCDLBCL-LT for six months. Violaceous nodules were the second symptom and they progressively developed on the edematous calf. Initially, they were diagnosed as warty overgrowths, which are common skin changes in the course of chronic lymphedema. The lack of improvement in the violaceous nodules after compression therapy prompted to perform a skin biopsy. Histopathological evaluation revealed the presence of PCDLBCL-LT. In this article, we want to highlight the challenges of making a diagnosis of PCDLBCL-LT.To our knowledge, no other study has reported on lymphedema as an initial symptom of PCDLBCL-LT.