title: Monozygotic twins concordant for intestinal Behçet's disease.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Toyohashi Municipal Hospital, Toyohashi, Japan.
authors.forename: Taku
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Sudo
authors.forename: Shozo
authors.initials: S
authors.lastname: Okamura
authors.forename: Shinji
authors.initials: S
authors.lastname: Ohashi
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Urano
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Hosoi
authors.forename: Kose
authors.initials: K
authors.lastname: Segawa
authors.forename: Nobuhisa
authors.initials: N
authors.lastname: Mizuki
authors.forename: Masao
authors.initials: M
authors.lastname: Ota
mesh_terms: D000293:Adolescent; D000483:Alleles; D001528:Behcet Syndrome / Q000097:blood / Q000235:genetics / Q000473:pathology*; D001706:Biopsy; D003093:Colitis, Ulcerative / Q000097:blood / Q000235:genetics / Q000473:pathology*; D003113:Colonoscopy; D004247:DNA / Q000032:analysis; D003937:Diagnosis, Differential; D018450:Disease Progression; D004200:Diseases in Twins; D005819:Genetic Markers; D015235:HLA-B Antigens / Q000235:genetics; D059949:HLA-B51 Antigen; D015395:Histocompatibility Antigens Class I / Q000235:genetics; D006801:Humans; D008297:Male; D010641:Phenotype; D016133:Polymerase Chain Reaction; D014430:Twins, Monozygotic
chemical_list: D005819:Genetic Markers; D015235:HLA-B Antigens; D059949:HLA-B51 Antigen; D015395:Histocompatibility Antigens Class I; C099187:MHC class I-related chain A; D004247:DNA