Toxic Epidermal Necrolysis, Agranulocytosis and Erythroid Hypoplasia associated with Sulphasalazine

Case report A 39-year-old brick moulder was admitted on 13 April 1977 with a six-week history of abdominal pain, diarrhoea and loss of three stones in weight. His bowels were opened six times daily and he passed fresh blood and considerable mucus in his stools. On examination he was wasted, febrile (temperature 38.3°C) and fluid depleted. There was central abdominal tenderness and guarding. Sigmoidoscopy revealed an ulcerated bleeding rectal mucosa and rectal biopsy confirmed an active proctitis consistent with ulcerative colitis. A barium enema examination showed diffuse ulceration with pseudopolyp formation throughout the whole colon. No pathogens were isolated from his stools and the Widal reaction was negative. He denied taking any drugs before or during his illness and had no history of previous drug allergies or other allergic conditions. Treatment was commenced with intravenous fluids, intravenous hydrocortisone and oral prednisolone. He also received oral sulphasalazine I g twice daily for 4 days and 2 g 4 times daily for 27 days. On admission the patient's haemoglobin was 13.5 gldl but by 10 May 1977 it had fallen to I\.2 gldl with a reticulocyte count of 7%. Haematological tests showed no evidence of haemolysis including an absence of Heinz bodies, negative Coombs' and Schumm's tests, and normal plasma bilirubin, haptoglobin and urinary urobilinogen concentrations. Spleen size and function were normal using the 51-chromium labelled heat-damaged red cells test. The fall in haemoglobin was considered to be due to blood loss, fluid repletion