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

腿型原發性皮膚大B細胞淋巴瘤以慢性潰瘍表現之罕見個案報告

袁聖哲(Ted Sheng-Che Yuan) ； 李建智(Jian-Jr Lee) ； 宋詠娟(Yung-Chuan Sung) ； 黃世鴻(Shih-Hung Huang) ； 劉致和(Charles Chih-Ho Liu) ； 陳明庭(Ming-Ting Chen)

《臺灣整形外科醫學會雜誌》 21卷3期 (2012/09) Pp. 242-249

https://doi.org/10.7096/TJTSPS.201209.0242

摘要

背景：原發性皮膚B細胞淋巴瘤（PCBCL）是指在沒有其他皮膚外疾病的證據下之罕見皮膚B細胞淋巴瘤。在世界衛生組織-歐洲癌症研究與治療組織（WHO-EORTC）的分類，主要的亞型包含原發性皮膚邊緣區B細胞淋巴瘤（PCMZL）、原發性皮膚濾泡中心性淋巴瘤（PCFCL）以及腿型原發性皮膚大B細胞淋巴瘤（PCLBCL，leg type）。腿型原發性皮膚大B細胞淋巴瘤是最罕見和最具侵略性的皮膚B細胞淋巴瘤。目標及主旨：藉由此報告分享這種罕見疾病的經驗，認識腿型原發性皮膚大B細胞淋巴瘤可能以慢性潰瘍來表現，而不是典型的藍色結節。材料及方法：此病人為33歲男性，主訴左大腿內側出現慢性潰瘍併發炎已半年。最初，腫脹的大腿有壓痛及逐漸形成皮膚潰瘍，並且對於抗生素的治療無反應。除了出血性潰瘍，左大腿外側粉紅色皮膚斑塊的存在使得被懷疑為惡性腫瘤。病理報告證實為侵略皮膚之瀰漫性大B細胞淋巴瘤。胸部X光、腹部超音波、骨骼掃瞄以及核磁共振並沒有發現皮膚以外的疾病。結果：經過八週期的化療（CHOP），腿型原發性皮膚大B細胞淋巴瘤達到緩解以及潰瘍達到全癒而無須皮膚移植。隨後八年追蹤未發現腫瘤復發。結論：腿型原發性皮膚大B細胞淋巴瘤是一種極為罕見的疾病，需要病理切片及排除非皮膚的疾病。在此個案中，它模仿慢性潰瘍併蜂窩組織炎。傷口的切片化驗是診斷原發性皮膚B細胞淋巴瘤的重要關鍵之一。藉由明確診斷此疾病和早期化療是主要的治療方式。

並列摘要

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)

並列關鍵字

primary cutaneous large B-cell lymphoma ； leg type ； chronic ulcer ； PCBCL ； PCLBCL