Ichthyosis and marrow involvement in malignant histiocytosis of the intestine

It has recently been suggested that a number of small bowel lymphomas arise from histiocytic cells in the intestine. This has been referred to as malignant histiocytosis of the intestine (MHI) (Isaacson et al, 1979). Three such patients with small bowel malabsorption had the following features: subtotal villous atrophy of the jejunum, generalized ichthyosis, fever and lymphopenia are described. In all three, the bone marrow appearances were similar to those seen in histiocytic medullary reticulosis (HMR) (Scott & Robb-Smith, 1939). A tumour was present in the small bowel and/or mesentery of all patients and the histological lesion was similar to that described as MHI. Two patients had a response to combination chemotherapy. One patient had a complete remission of his disease but this relapsed after 1 year and proved refractory to chemotherapy. A second patient died following chemotherapy with gastro-intestinal perforation and septicaemia and the third patient died shortly after diagnosis and before chemotherapy could be commenced. It is suggested that the combination of signs, symptoms and pathological features described may reflect a specific clinical entity which has not previously been described.