Abstract
A 58-year-old woman was diagnosed with systemic sclerosis and polymyositis in 1983. In February of 1999, she noticed a subcutaneous nodule in her right trunk and then developed fever, appetite loss and fatigue. Skin biopsy specimen revealed infiltrates of large atypical lymphocytes and histiocytes showing a “bean bag” appearance in dermis and subcutis. Immunohistochemical findings revealed B-cell lymphoma. Bone marrow showed similar cytophagia without atypical lymphocytes. We diagnosed her with malignant lymphoma associated with hemophagocytic syndrome (HPS). There are only 21 previous cases reported as B-cell lymphoma associated with HPS. Only two of them were related to skin lesions.
