title: Gastrointestinal Tract Involvement of Gorham's Disease with Expression of D2-40 in Duodenum.
abstract: We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.
authors.affiliation: Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Bong Seok
authors.initials: BS
authors.lastname: Choi
authors.affiliation: Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Suk Jin
authors.initials: SJ
authors.lastname: Hong
authors.affiliation: Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Mi Ae
authors.initials: MA
authors.lastname: Chu
authors.affiliation: Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Seok Jong
authors.initials: SJ
authors.lastname: Lee
authors.affiliation: Department of Radiology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Jong-Min
authors.initials: JM
authors.lastname: Lee
authors.affiliation: Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Han Ik
authors.initials: HI
authors.lastname: Bae
authors.affiliation: Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Byung-Ho
authors.initials: BH
authors.lastname: Choe
keywords: Child; Gastrointestinal tract; Gorham disease; Melena; Occult blood; Osteolysis
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