Lymphome cutané primitif diffus à grandes cellules B-type jambe- de localisation faciale: image en médecine

Abstract

Primary cutaneous diffuse large B-cell lymphoma, leg-type (PCDLBCL-LT) is a very rare, aggressive variant of extranodal lymphomas (accounting for 4% of all cutaneous lymphomas). It mainly affects women, with a median age of 76 years. As its name suggests, PCDLBCL-LT mainly occurs on the lower limbs. We here report the case of a 63-year-old male African patient presenting with multifocal nodules on his face that had been evolving for 3 months and whose appearance was primarily suggestive of cutaneous carcinoma, localized cutaneous granulomatosis or cutaneous metastasis. The diagnosis of PCDLBCL-LT was confirmed after skin biopsy with immunohistochemistry, showing positive B markers (CD20 and CD79a) associated with BCL2, BCL6 and Mum1 positivity and CD10 CD3 and CD5 negativity. The Ki67 proliferation index was estimated at 71% and Pet-scan did not show any other disease location. The patient received immunochemotherapy combining rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone, with good clinical improvement.