Abstract
Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade malignant B-cell lymphoma that originates from the skin. It often presents as erythematous solitary or multiple papules, nodules, and/or plaques. It is one of the three main subtypes of primary cutaneous B-cell lymphomas. PCMZLs are thought to develop from chronic antigenic stimulation such as from tick-borne bacteria, vaccines, tattoo pigment, or other foreign body. Additionally, cutaneous lymphoid hyperplasia (CLH), a documented precursor to malignant PCMZL, often presents in response to areas of chronic inflammation. Cutaneous lymphoid hyperplasia and PCMZL share several clinical and histological similarities that require clinicopathologic suspicion, immunohistochemical stains, and histopathologic analysis to accurately differentiate the two entities. While gene rearrangement studies have historically been of limited value in the diagnosis of PCMZL, recent studies investigating molecular markers have identified the presence of multiple genetic abnormalities that have helped to better characterize the disease and aid in diagnosis. In this article, we describe a case of a 56-year-old patient presenting with chronic reactive lymphoid hyperplasia that transformed to primary cutaneous marginal zone lymphoma.
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