Primary Cutaneous Large B-cell Lymphoma of Leg, with Unusual Presentation as a Chronic Ulcer-A Case Report

Abstract

Background: Primary cutaneous B cell lymphoma (PCBCL) refers to those rare cases of B-cell lymphoma that present in the skin without evidence of extracutaneous disease. In the World Health Organization-European Organization for the Research and Treatment of Cancer (WHO-EORTC) classification, the major subtypes are: primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous large B-cell lymphoma of leg (PCLBCL, leg type). PCLBCL, leg type is the most rare and aggressive form of PCBCL. Aim and Objectives: Our purpose is to share our experience of this rare disease and to bring awareness to the possibility of PCLBCL (leg type) presenting with chronic ulcer instead of bluish nodules.Materials and Methods: This case report describes a 33 year-old male with presentation of left medial thigh chronic ulcer and cellulitis for 6 months. Initially, the swollen leg with progressive tenderness and huge ulcerations were observed and had no response to antibiotic treatment. Besides bleeding ulcers, the presence of pinkish skin plaques raised suspicion of malignancy. Pathology revealed diffuse large B-cell lymphoma involving the dermis. Plain chest film, sonography, bone scan and MRI did not show extracutaneous manifestations. Results: After multiagent chemotherapy with eight cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), PCLBCL of leg achieved a complete response and the ulceration healed without skin graft. Follow-up of 8 years showed no tumor relapse.Conclusion: Primary cutaneous large B-cell lymphoma of leg is an extremely rare disorder which requires biopsy of involved skin and thorough study to exclude non-cutaneous disease. As in this case, it mimics chronic ulcer and cellulitis at presentation. It is always crucial to perform biopsy on chronic ulcers. Given the aggressive nature of this disease, early diagnosis and chemotherapy is appropriate treatment modalities. (J Taiwan Soc of Plast Surg 2012;21:242~249)