Monozygotic twins concordant for intestinal Behçet's disease.

Although Behçet's disease (BD) is a multisystem disorder of unknown causes, both genetic and environmental factors have been suggested. This is the second reported case of monozygotic twins concordant for Behçet's disease and the first such report of intestinal Behçet's disease. Patient 1 was a 17-year-old man with fever, recurrent oral aphthae, and skin eruptions. He developed hematochezia and was given corticosteroid empirically. One month after he was discharged, he again developed oral ulcerations, fever, and hematochezia. Colonoscopy was performed again, showing aphthous ulcerations in the entire colon, and deep oval ulcers with marginal elevation around the ileocecal valve, which are characteristics of intestinal Behçet's disease. He was treated with colchicine and azathioprine in combination with salazosulfapyridine (SASP) and prednisolone (PSL) and achieved remission. Patient 2 was the twin brother of patient 1. He was admitted because of oral aphthous ulcerations, fever, pustules on his face and body, and genital ulcers. Two weeks later he developed hematochezia. Colonoscopic and barium enema findings were similar to those of his brother. SASP, PSL, colchicines, and azathioprine were also required to achieve remission. Both of the patients were diagnosed with intestinal Behçet's disease. Their monozygosity was confirmed by detailed genetic typing, and HLA-B51 was negative.

Efficacy of cyclosporin with corticotropin for refractory ulcerative colitis.

BACKGROUND/AIMS: Cyclosporin was reported to be useful for steroid-resistant severe ulcerative colitis in the short term, but limited data are available on the long-term follow-up of such patients. Our aim was to assess the short- and long-term efficacy of combination therapy with cyclosporin and corticotropin for steroid-resistant ulcerative colitis. METHODOLOGY: Twenty-one patients with ulcerative colitis who did not respond to corticosteroid therapy, were treated with corticotropin, and 9 patients (43%) of them achieved complete remission. Twelve patients (57%) who did not achieve complete remission by corticotropin alone were given combination therapy with cyclosporin and corticotropin. RESULTS: In 12 patients who received combined therapy with cyclosporin and corticotropin, clinical severity was distinctly improved in 11 patients (92%) by combination therapy within 2 weeks and 7 patients (58.3%) entered into complete remission with salicylazosulfapyridine or 5-aminosalicylic acid alone. Two patients (16.7%) demonstrated insufficient effect and continue to receive a lower dosage of cyclosporin or corticosteroid. Three patients (25%) failed to respond to the combination therapy and required colectomy. Three of 7 patients who entered into remission relapsed 0.5, 5 and 5.5 months (average: 3.7 months) after cyclosporin withdrawal, but the clinical severity at the time of relapse was milder than that at the beginning of the treatment, namely, moderate in 2 patients, and mild in 1 patient. There were no significant adverse effects in our series. CONCLUSIONS: We demonstrated that oral cyclosporin in combination with corticotropin was highly effective for ulcerative colitis refractory to corticosteroid or corticotropin therapy and severe relapse was uncommon during several years of follow-up.

[Is p53 immunohistochemistry useful for optimal decision for treatment of polypoid lesions in ulcerative colitis?].

BACKGROUND: Usefulness of p53 staining for the differentiation between adenoma and DALM has been reported recently, so recognizable lesions stained positively can be diagnosed as DALMs. For the cases with DALMs, total colectomy has been thought to be necessary. METHODS: Immunohistochemical staining for p53 was performed in 4 adenocarcinomas and 4 adenomas in ulcerative colitis. RESULTS: Three carcinomas and 3 adenomas were positive. One carcinoma (protruded mucosal cancer) and 3 adenomas (1 flat elevated lesion and 2 laterally spreading tumors) stained positively for p53 were treated only by polypectomy or local excision. The patients have been under surveillance for periods ranging from 1 to 10 years, during which no metachronous dysplasia has developed. CONCLUSIONS: These findings suggest that some groups of the polypoid lesions can be resected locally even if stained positively by p53 immunohistochemistry.