A unique case of extranodal marginal zone lymphoma with synchronous pulmonary and dermatologic manifestations

An 89-year-old male with a medical history of non-ischemic cardiomyopathy was initially admitted with acute hypoxic respiratory failure attributed to heart failure exacerbation. Aside from progressive dyspnea, a non-pruritic, non-painful rash and constitutional symptoms were reported. Initial work-up was remarkable for normocytic anemia, lymphopenia, mild hypercalcemia, and elevated inflammatory markers. Despite aggressive diuresis, his respiratory distress worsened requiring up-titration of supplemental oxygen (6-8L/min). Subsequent chest CT showed diffuse, ill-defined areas of consolidation and ground-glass opacities (GGOs) with areas of solid and ground-glass nodularity. Rheumatologic work-up was remarkable for mildly elevated ANA titer of 1:60, and positive anti-centromere antibody of 1.8 AI (normal range 0-0.9 AI). Infectious work-up was negative. Due to high oxygen requirements, tissue sampling was obtained by skin biopsy instead of bronchoscopy. After biopsy testing, prednisone 60 mg was started with posterior clinical and radiographic improvement. Biopsy results revealed cutaneous MZL. Follow-up PET scan showed persistent but improved diffuse GGOs and nodular opacities. Given the clinical presentation, imaging and skin biopsy results, the diagnosis was compatible with EMZL with synchronous pulmonary and skin manifestations. Empiric treatment with Rituximab and steroid taper was planned. At 6-month follow-up, the patient reported clinical and respiratory improvement.