Primary Cutaneous Marginal Zone Lymphoma: An Interesting Presentation

Abstract

Primary cutaneous marginal zone lymphoma (PCMZL) is a subtype of extranodal marginal zone B-cell lymphoma which may involve mucosal sites. Approximately 8% of non-Hodgkin lymphomas can be classified as marginal zone lymphomas. PCMZL presents more frequently in the fifth and sixth decades as red papules, plaques, or localized nodules typically located on the trunk, arms, or head. Male patients present twice as often as female patients, and the median age is 55 years old. Diagnosis of PCMZL is made by skin biopsy, containing reticular dermis and fat, which can assist in distinguishing it from a reactive or inflammatory process. By definition, a primary cutaneous lymphoma affects the skin without evidence of disease elsewhere in the body when diagnosed. Marginal zone lymphoma (MZL) can include mucosal sites and extend to the lymph nodes and bone marrow, making MZL disseminated in approximately one-third of patients. Treatment of PCMZL includes surgery, radiotherapy, and topical therapy. The five-year overall survival rate for PCMZL is estimated to be 96.6% with a 10-year overall survival of 90.5%. A 70-year-old female presented for a facial rash present for 4 months. The patient reported the rash started as a small, pruritic, erythematous patch. The patch later evolved into an erythematous plaque covering bilateral cheeks and extending to the upper cutaneous lip. The results of the biopsy were consistent with primary cutaneous marginal zone lymphoma. The case is of interest because of the acute presentation in an older female with facial lesions only.