Extranodal lymphomas are lymphomas that evolve in nonlymphoid tissue, such as the gastrointestinal tract and skin. There are three main types of primary cutaneous lymphomas: primary cutaneous follicular center lymphoma (PCFCL); primary cutaneous large B-cell lymphoma (PCLBCL), leg type; and primary cutaneous marginal zone lymphoma (PCMZL).1
Primary cutaneous diffuse large B-cell lymphoma, leg type, is an extranodal, intermediate-grade B-cell lymphoma.1-3 It comprises 1% to 4% of all cutaneous lymphomas and up to 20% of primary cutaneous B-cell lymphomas. There are no clear risk factors or hereditary tendencies. The incidence is approximately 0.5 to 1 case per 100,000 persons annually. It occurs more in females than males (ratio 1:3-4) and the median age is mid- to late-70s. It usually presents as red or bluish nodules on the lower extremities, but can occur in other locations. The typical “B” symptoms associated with lymphoma (fever, chills, night sweats, weight loss, fatigue) are usually not present. The diagnosis is confirmed by punch or excisional biopsy. The cells usually show a diffuse infiltrate of large B cells, centroblasts, or immunoblasts. Immunohistochemical stains are often positive for bcl-2 protein, Mum1, and FOXP1.1-3
Five-year survival rates have been reported as 41% to 66%. The presence of multiple skin lesions at diagnosis is a worse prognostic factor.4 Treatment consists of anthracycline-based chemotherapy regimens with rituximab and possible radiation therapy.2
Aileen Anglin is a nurse practitioner with Lake Physician Group Medical Oncology in Baton Rouge, Louisiana.
1. Jacobsen E, Freedman AS, Willemze R. Primary cutaneous large B cell lymphoma, leg type. UpToDate Web site. http://www. uptodate.com/contents/primary-cutaneous-large-b-cell-lymphoma-leg-type. This topic last updated January 5, 2015. Accessed June 23, 2015.
2. Sokol L, Naghashpour M, Glass LF. Primary cutaneous B-cell lymphomas: recent advances in diagnosis and management. Cancer Control. 2012;19(3):236-244.
3. Thomas V, Dobson R, Mennel R. Primary cutaneous large B-cell lymphoma, leg type. Proc (Bayl Univ Med Cent). 2011;24(4):350-353.
4. Grange F, Beylot-Barry M, Courville P, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol. 2007;143(9):1144-1150.