Sultan Qaboos University Med J, November 2014, Vol. 14, Iss. 4, pp. e587−588, Epub. 14TH Oct 14 Submitted 14TH Feb 14 Revision Req. 1ST Apr 14; Revision Recd. 1ST Apr 14 Accepted 17TH Apr 14 Departments of 1Dermatology and 2Pathology, Complejo Hospitalario de Jaen, Jaen, Spain *Corresponding Author e-mail: ismenios@hotmail.com سرطان الغدد الليمفاوية ذو اخلاليا B الكبرية املنتشر تشخيص ال جيب نسيانه يف كبار السن ريكاردو رويز- فيالفريدي وفران�سي�سكو رامو�س-بلجيوزيولو�س Diffuse Large B-cell Lymphoma, Leg Type A diagnosis not to forget in the elderly *Ricardo Ruiz-Villaverde1 and Francisco Ramos-Pleguezuelos2 INTERESTING MEDICAL IMAGE Figure 1: Infiltrated erythematous nodules on the anterior aspect of the left leg with severe hyperkeratosis and secondary impetiginisation. An 83-year-old woman, with a past medical history of hypertension, diabetes mellitus and ischemic cardiomyopathy, was referred to the dermatology clinic of Complejo Hospitalario de Jaen in Jaen, Spain, in October 2013. The patient presented with erythematous indurated tumoural lesions with severe hyperkeratosis and impetiginisation on the anterior aspect of her left leg, which had appeared eight months prior [Figure 1]. Fever, night sweats, weight loss, itching or other constitutional symptoms were absent. Neither hepatosplenomegaly nor axillary or generalised lymphadenopathy were detected. A systemic examination of the patient did not reveal any further abnormalities. All laboratory tests were negative, including a full blood count; biochemistry, protein electrophoresis, routine urine, lactate dehydrogenase, β2- microglobulin and immunoglobulin tests; a peripheral blood smear, and serology tests for viruses (human immunodeficiency virus and hepatitis B and C viruses) and Borrelia burgdorferi. No evidence of malignancy was observed during a whole-body computed tomography scan or a bone marrow biopsy. A cutaneous biopsy was also performed. A histological examination of the skin specimen revealed a B-lymphocyte proliferation with mild epidermotropism and antigen CD20/45 positivity. The following profile was obtained from an immunohistochemical assay: positive multiple myeloma oncogene 1 (MUM-1), positive B-cell lymphoma 6 (Bcl-6), negative chromosome 10 (C10), positive B-cell lymphoma 2 (Bcl-2) and antigen Ki67 >90%. These findings were consistent with a diagnosis Figure 2: B-lymphocyte inflitration with a predominance of medium size and split non-cleaved cells with blastic differentiation. Diffuse Large B-cell Lymphoma, Leg Type A diagnosis not to forget in the elderly 588 | SQU Medical Journal, November 2014, Volume 14, Issue 4 of diffuse large B-cell lymphoma, leg type [Figure 2]. In addition, testing for the Epstein-Barr virus by in situ hybridisation was negative. Comment Diffuse large B-cell lymphoma, leg type is a subset of primary cutaneous lymphoma, characterised by the presence of nodules or tumours on the legs of elderly people, mainly females.1 It represents 1–4% of all cutaneous lymphomas and has a five-year survival rate of less than 50%.2 This form of lymphoma was identified as an independent entity in the classification of cutaneous lymphomas developed by the European Organisation for Research and Treatment of Cancer.3 Although these lymphomas are mostly limited to the skin at presentation, in 10% of reported cases the tumoural lesions begin in locations other than the legs.4 When the malignancy spreads, it frequently advances to the lymph nodes, bone marrow and the central nervous system.4 As the majority of the patients with this condition are elderly, the therapeutical management may be complicated due to comorbidities and the potentially frequent number of relapses. Spontaneous remission only occurs in rare cases.5 Rituximab plus cyclophosphamide, doxorubicin, vincristine and prednisolone (R-CHOP) chemotherapy and chemotherapeutic compounds applied for relapsing systemic B-cell non-Hodgkin’s lymphoma have previously proven to be effective therapies.6 However, the current first line of treatment for this variety of lymphoma involves anti-CD20 antibodies, such as rituximab. These therapies can be used even during the early stages of the condition in order to improve rates of survival.7 References 1. Hernández-Machín B, Fernández-Misa R, Alfonso JL, Maeso MC, Marrero C, Borrego L. [Primary cutaneous diffuse large B-cell lymphoma of the leg according to the new WHO- EORTC classification: Two cases]. Actas Dermosifiliogr 2005; 96:607–11. 2. Suárez AL, Pulitzer M, Horwitz S, Moskowitz A, Querfeld C, Myskowski PL. Primary cutaneous B-cell lymphomas: Part I - Clinical features, diagnosis, and classification. J Am Acad Dermatol 2013; 69:329. doi: 10.1016/j.jaad.2013.06.012. 3. 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. doi: 10.1182/blood-2004-09-3502. 4. Grange F, Bekkenk MW, Wechsler J, Meijer CJ, Cerroni L, Bernengo M, et al. Prognostic factors in primary cutaneous large B-cell lymphomas: a European Multicenter Study. J Clin Oncol 2001; 19:3602–10. 5. Alcántara-González J, González-García C, Fernández-Guarino M, Jaén-Olasolo P. Spontaneous regression of primary diffuse large B-cell lymphoma, leg type. Actas Dermosifiliogr 2014; 105:78–83. doi: 10.1016/j.ad.2012.07.009. 6. Ekmekci TR, Koslu A, Sakiz D, Barutcuoglu B. Primary cutaneous large B-cell lymphoma, leg type, presented with a migratory lesion. J Eur Acad Dermatol Venereol 2007; 21:1000– 1. doi: 10.1111/j.1468-3083.2006.02073.x. 7. Gopal MM, Malik A. Primary cutaneous diffuse large B-cell lymphoma of the upper limb: A fascinating entity. Indian J Dermatol 2013; 58:366–8. doi: 10.4103/0019-5154.117303.