Abstract
We report a case of a 30-year-old female with a six-month history of shortness of breath, malaise, and a cutaneous eruption. Given the progression of pulmonary symptoms and cutaneous eruption, she was admitted for further work up and treatment. Imaging revealed bilateral pneumonia and splenomegaly. A lung biopsy was performed which showed organizing acute lung injury with granulomatous features and a T cell infiltrate. Skin biopsies were obtained which revealed an atypical lymphohistiocytic and granulomatous infiltrate with rare scattered CD20-positive lymphocytes. Epstein Barr virus (EBV) positivity was detected by in situ hybridization (ISH) for EBV-encoded RNA (EBER) containing cells. T-cell receptor (TCR) gene rearrangement studies were negative. The patient was treated with oral corticosteroids with some improvement of her shortness of breath, however her cutaneous disease continued to progress, with development of deep-seated nodules on the abdomen. Repeat biopsies were obtained which demonstrated a similar atypical lymphohistiocytic and granulomatous infiltrate with angiocentricity and many CD20-positive lymphocytes demonstrating EBER ISH positivity (grade 2). These findings were consistent with a diagnosis of lymphomatoid granulomatosis (LyG). Lymphomatoid granulomatosis is a rare lymphoproliferative disease driven by EBV. Clinically, it typically involves the lung, though it may also involve the skin, kidney, liver, and other organs. Histology shows EBV positive atypical B cells and angioinvasive T-cell infiltrate with necrosis. The pathogenesis of this condition is thought to be due to defective immune surveillance secondary to EBV infection. Treatments for this condition are varied and include systemic corticosteroids and immunochemotherapy.
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