Meziane M, Hesse S, Chetaille B, Bien-Aimée A, Grob J J, Richard M A
Service de dermatologie, CHU de La Timone, 264, rue Saint-Pierre, 13385 Marseille cedex 5, France.
Ann Dermatol Venereol. 2009 Nov;136(11):791-4. doi: 10.1016/j.annder.2009.02.005. Epub 2009 Jun 26.
Primary cutaneous B-cell lymphomas form a heterogeneous group of lymphoid proliferations found on the skin. We report a case of primary leg-type cutaneous large B-cell lymphoma occurring on the site a previous leg burn. A few rare cases of cutaneous lymphoma forming on burn scars have been described, but these concern primary cutaneous lymphomas of the T-cell phenotype.
An 85-year-old man with a history of a burn to the left leg 17 years ago, previously treated with several skin grafts, presented numerous ulcerative budding lesions on the scar area. Histological examination of the skin biopsy revealed the existence in the skin ulcers of atypical large lymphoid cells having an immunoblastic or centroblastic morphology and shown by immunohistochemistry to be of the B-cell phenotype, thereby evoking a diagnosis of large B-cell lymphoma. The lymphoma cells were positive for MUM1/IRF4 and BCL2, and more weakly for BCL6, but negative for CD10. The staging examination revealed only cortical lysis of the left tibia. Temporary initial regression was achieved by polychemotherapy comprising cyclophosphamide, vincristine and prednisone in combination with rituximab.
This case is novel in that it involves primary large B-cell lymphoma, leg type, occurring on burn scar tissue. Venous insufficiency and lymphatic stasis have already been incriminated in the genesis of this type of lymphoma; the prior injury and resulting immune dysregulation at the burn site may have also contributed to the development of this neoplasia.
原发性皮肤B细胞淋巴瘤是一组在皮肤上发现的异质性淋巴增殖性疾病。我们报告一例原发性腿部型皮肤大B细胞淋巴瘤发生于先前腿部烧伤部位。已有少数关于在烧伤瘢痕上形成皮肤淋巴瘤的罕见病例报道,但这些均为T细胞表型的原发性皮肤淋巴瘤。
一名85岁男性,17年前有左腿烧伤史,曾接受多次皮肤移植,在瘢痕区域出现大量溃疡性芽状病变。皮肤活检的组织学检查显示,皮肤溃疡中存在具有免疫母细胞或中心母细胞形态的非典型大淋巴细胞,免疫组化显示为B细胞表型,从而提示诊断为大B细胞淋巴瘤。淋巴瘤细胞MUM1/IRF4和BCL2呈阳性,BCL6呈弱阳性,但CD10呈阴性。分期检查仅发现左胫骨皮质溶解。通过环磷酰胺、长春新碱和泼尼松联合利妥昔单抗的多药化疗实现了暂时的初始缓解。
该病例的新颖之处在于它涉及发生于烧伤瘢痕组织的原发性腿部型大B细胞淋巴瘤。静脉功能不全和淋巴淤滞已被认为与这类淋巴瘤的发生有关;先前的损伤以及烧伤部位由此导致的免疫失调可能也促成了这种肿瘤的发展。