Extensive alimentary and genital ulceration, mesenteric cysts, malabsorption, T-lymphocyte depletion and subsequent anaplastic bladder carcinoma.

Case report A male printer born in 1937 was first seen in January 1971 complaining of dyspepsia. A barium meal examination was negative and he was treated symptomatically. In March 1973 he complained of loss of appetite, difficulty in swallowing with food appearing to stick at various levels, and a weight loss from about 66 to 60 kg over the previous few months. A month later he developed severe mouth ulcers which were noted to be extensive, confluent and yellowish in colour. At the same time he developed ulcers on the glans and shaft of the penis. A repeat barium meal was normal and subsequently a barium swallow was also normal. Oesophagoscopy showed multiple superficial ulcers at all levels, and the appearance was unlike peptic oesophagitis. Two months later he was referred to a dermatology clinic with scaly discrete skin lesions on his trunk. A skin biopsy showed hyperplasia, dyskeratosis, spongiosis, and microvesiculation with endothelial swelling, with neutrophil and lymphocyte infiltration similar to pityriasis lichenoides et varioliformis. It was considered that he had the minor mucocutaneous variety of Behqet's disease. In August 1973 he complained of diarrhoea for the first time, with frequent pale offensive stools which were difficult to flush away, and his weight had fallen again. His dysphagia and dyspepsia were controlled with oral hydrocortisone pellets and an antacid containing local anaesthetic. By February 1974 he felt considerably better with improved appetite and his weight was increasing. The improvement led to loss from follow-up. He was referred to a general medical clinic 2 years later complaining again of dyspepsia. He also had diarrhoea with the character of steatorrhoea and further weight loss to 57 kg. On examination there was a vague inconstant left-sided abdominal swelling. Oral ulcers persisted but there were no genital ulcers. As his weight fell to 51 kg over the next 3 months he was admitted to hospital for full evaluation. Investigations carried out at this time included the following: sigmoidoscopy showed pale stool with only a mild mucosal erythema; histology confirmed a mild non-specific proctitis; barium enema was normal, but barium meal and follow-through demonstrated abnormality throughout the entire small intestine and also in the right colon, with extensive loss of mucosal pattern; upper digestive endoscopy revealed severe oesophagitis with oesophageal ulcers, duodenal ulcers and evidence of a chronic gastritis; the peak acid output was normal at 33-2 mmol/hr; alimentary histology showed a severe inflammatory infiltrate in the oesophagus and chronic gastritis; jejunal biopsy histology showed subtotal villous atrophy with superficial erosions and a heavy plasma cell infiltrate into the submucosa.