Lyell's syndrome and lymphoma

The authors present the case of a patient affected by Lyell's Syndrome and Lymphoma that was revealed in the post-mortem examination. At onset the disease showed mucosal hyperaemia, oedema and erosions. In about ten days macular lesions with vesciculation and bullae appeared on the abdomen. General conditions did not seem to be affected. Three weeks later a fever was noted. No important illness, nor drug assumption were detectable in the history of the patient. At the beginning of the fourth week a dramatic worsening occurred with erythrodermic state and high fever. Death occurred at the sixth week in spite of therapy. Bacteriological and virological examinations were always negative. Post-mortem examination showed retroperitoneal and pelvic neoplastic tissue, involving lumboaortic lymph nodes. Histopathology demonstrated a low grade malignancy non Hodgkin Lymphoma, diffused type with small lymphocytes showing plasmocytic differentiation. The lack of drug intake or infectious disease in the history, underlines the peculiar association between the tumor and the syndrome. Rare cases of TEN associated with lymphoproliferative neoplasm are reported in the literature, and the pathogenetic problem of the syndrome is reviewed.