Primary Cutaneous Diffuse Large B Cell Lymphoma Leg Type. An Atypical Presentation

Keywords: Lymphoma, Large B-Cell, Diffuse/diagnosis, Lymphoma, Large B-Cell, Diffuse/therapy, Skin Neoplasms


Primary cutaneous lymphomas are defined as a heterogeneous group of malignant lymphoproliferative neoplasms that attack the skin, without extracutaneous involvement at the moment of the diagnosis. The subgroup "primary cutaneous lymphoma of great cells B, leg type (PCLBCL, LT)" generally attacks one or both lower limbs, however in 10% - 15 % of the cases other areas of the skin may be affected. We present a rare case of PCLBCL, LT with a nodular lesion located in the cervical region to empathize an atypical presentation. Considering its quick growth and high proliferative rate, it is of great importance to know all its possible clinical presentations for a precocious diagnosis and efficient treatment.


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Hope CB, Pincus, LB. Primary cutaneous b-cell lymphomas. Clin Lab Med. 2017; 37: 547-74.

Sousa AR, Filho EF, Miranda WL, Costa IS, Jucá NB. Primary cutaneous large B-cell lymphoma of atypical presentation - Case report. An Bras Dermatol. 2011; 86:549-51.

Marrero-Alemán G, Montenegro-Dámaso T, Peñate Y. Primary cutaneous diffuse large B-cell lymphoma, leg type, with spontaneous regression after biopsy. Am J Dermatopathol. 2017; 39: 785-7. doi: 10.1097/DAD.0000000000000874.

Wilcox RA. Cutaneous B-cell lymphomas: 2019 update on diagnosis, risk stratification, and management. Am J Hematol. 2018; 93: 1427–30. doi: 10.1002/ajh.25224.

Vermeer MH, Willemze R. Recent advances in primary cutaneous B-cell lymphomas. Curr Opin Oncol. 2014; 26: 230-6. doi: 10.1097/CCO.0000000000000060.

Osada S, Fujiwara Y, Higashi N, Nakayama K, Hosone M, Inokuchi K et al. Primary cutaneous diffuse large B cell lymphoma, leg type, localized in the right periorbital region. J Dermatol. 2018 ;46(4):e130-1. doi: 10.1111/1346-8138.14657.

Goyal A, Carter JB, Barnes JA, Duncan LM. Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type. In: Carter J, Goyal A, McDivitt Duncan L, editors. Atlas of Cutaneous Lymphomas. Berlin: Springer; 2015.

Patsatsi A, Kyriakou A, Karavasilis V, Panteliadou K, SotiriadisD. Primary cutaneous diffuse large B-cell lymphoma, leg type, with multiple local relapses: case presentation and brief review of literature. Hippokratia. 2013; 17: 174-6.

KhatibY, Dande M, Patel RD, Makhija M. Primary cutaneous large B-cell lymphoma of scalp: Case report of a rare variant. Indian J Pathol Microbiol. 2017;60:268-71. doi: 10.4103/0377-4929.208414.

Pinter-Brown LC. Diagnosis and management of cutaneous B-cell lymphoma. Dermatol Clin. 2015; 33: 835-40.

Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019;133:1703-14. doi: 10.1182/blood-2018-11-881268.

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri AS, Stein H, et al. WHO classification of Tumors of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon: International Agency for Researchon Cancer; 2017.

Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768-85.

Sokol L, Naghashpour M, Glass F. Primary cutaneous B cell lymphomas: recent advances in diagnosis and management. Cancer Control. 2012; 19:236-44.

Thomas V, Dobson R, Mennel R. Primary cutaneous large B cell lymphoma, leg type. Proc. 2011; 24:350-3.

Hristov AC. Primary cutaneous diffuse large B-cell lymphoma, leg type. Arch Pathol Lab Med. 2012; 13:876-81.

Senff NJ, Hoefnagel JJ, Jansen PM, Vermeer MH, Baarleen JV, Blokx WA, et al. Reclassification of 300 Primary Cutaneous B-Cell Lymphomas According to the New WHO–EORTC Classification for Cutaneous Lymphomas: Comparison With Previous Classifications and Identification of Prognostic Markers. J Clin Oncol. 2007; 25:1581-7.

Pham-Ledard A, Cappellen D, Martinez F, Vergier B, BeylotBarry M, Merlio JP. MYD88 somatic mutation is a genetic feature of primary cutaneous diffuse large B-cell lymphoma, leg type. J Invest Dermatol. 2012; 132:2118-20. doi: 10.1038/jid.2012.102.

Pham-Ledard A, Beylot-Barry M, Barbe C, Leduc M, Petrella T, Vergier B, et al. High frequency and clinical prognostic value of MYD88 L265P mutation in primary cutaneous diffuse large B-cell lymphoma, leg-type. JAMA Dermatol. 2014; 150:1173-9. doi: 10.1001/jamadermatol.2014.821.

Toberer F, Mechtersheimer G, Jaschinski H, Enk A, Hakim-Meibodi L, Haenssle HA. Spontaneous regression of primary cutaneous diffuse large B-cell lymphoma, leg type. Acta Derm Venereol. 2018; 98: 608-9. doi: 10.2340/00015555-2921.

Jimura N, Fujii K, Baba A, Higashi Y, Kanekura T. Spontaneous regression of a primary cutaneous diffuse large B-cell lymphoma, leg type. J Dermatol. 2017;44: 608-9. doi: 10.1111/1346-8138.13496.

Kempf W, Denisjuk N, Kerl K, Cozzio A, Sander C. Primary cutaneous B-cell lymphomas. J Dtsch Dermatol Ges. 2012; 10:12-22. doi: 10.1111/j.1610-


Zinzani PL, Quaglino P, Pimpinelli N, Berti E, Baliva G, Rupoli S, et al. Prognostic factors in primary cutaneous B-cell lymphoma: The Italian Study Group for Cutaneous Lymphomas. J Clin Oncol. 2006; 24:1376-82.

Grange F, Beylot-Barry M, Courville P, Maubec E, Bagot M, Vergier B, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases. Arch Dermatol. 2007;143:1144-50.

Lima M. Cutaneous primary B-cell lymphomas: from diagnosis to treatment. An Bras Dermatol. 2015; 90:687- 706.

How to Cite
Sousa, N. A., Buffara, M. O., Maia, R. L., Pessoa, A. S., Cassia, F., & Brotas, A. M. (2020). Primary Cutaneous Diffuse Large B Cell Lymphoma Leg Type. An Atypical Presentation. Journal of the Portuguese Society of Dermatology and Venereology, 78(1), 61-65.
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