Michelle D. Sim, Claribel L Jimenez
Primary Cutaneous B-cell lymphomas represent 20%-30% of all cutaneous neoplasm. It has an estimated incidence of 10 case per 1,000,000 persons per year based on surveillance epidemiology. Primary Cutaneous Marginal Zone Lymphoma (PCMZL) is a rare and indolent type of cutaneous B-cell lymphoma which accounts for 10% of cases, with an excellent survival rate in 5 years exceeding 95%. It is composed of mixed infiltrates of marginal zone B-cells, lymphoplasmacytic cells, plasma cells, and reactive T-cells. We report a case of PCMZL in a 39 year old female who presented with generalized erythema, multiple violaceous plaques and nodules, located on the face, trunk and extremities. Definitive diagnosis based on clinicopathologic correlation as well as confirmation by immunohistochemical staining, is critical in distinguishing PCMZL from other types of lymphomas so that patients are properly evaluated and treatment management can be correctly instituted.