{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T01:54:58Z","timestamp":1782266098231,"version":"3.54.5"},"reference-count":22,"publisher":"American Academy of Pediatrics (AAP)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1999,6,1]]},"abstract":"<jats:sec>\n                  <jats:title>Background.<\/jats:title>\n                  <jats:p>Behc\u0327et's syndrome is a chronic relapsing vasculitis characterized by aphthous stomatitis, genital ulcerations, and uveitis. The disease is a multisystem illness, which may involve the skin, joints, gastrointestinal tract, blood vessels, central nervous system, and other organs.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case Study.<\/jats:title>\n                  <jats:p>We report a case of an infant with features of Behc\u0327et's syndrome who did not respond to conventional immunosuppression with high-dose corticosteroids and cytotoxic agents. Thalidomide, a potent immune response-modifying drug, was used with marked improvement and resolution of symptoms.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion.<\/jats:title>\n                  <jats:p>This finding supports a trial of thalidomide treatment in patients with Behc\u0327et's syndrome who are unresponsive to other forms of immunosuppressive therapy or develop undesirable side effects.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1542\/peds.103.6.1295","type":"journal-article","created":{"date-parts":[[2004,8,13]],"date-time":"2004-08-13T23:45:45Z","timestamp":1092440745000},"page":"1295-1297","source":"Crossref","is-referenced-by-count":29,"title":["Thalidomide Responsiveness in an Infant With Behc\u0327et's Syndrome"],"prefix":"10.1542","volume":"103","author":[{"given":"Lynette Pei-Chi","family":"Shek","sequence":"first","affiliation":[{"name":"Departments of Pediatrics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoke Sun Lee","family":"MD","sequence":"additional","affiliation":[{"name":"Pathology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bee Wah","family":"Lee","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas Joseph Anjorge","family":"Lehman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[1999,6,1]]},"reference":[{"key":"2021121002525309200_B1","first-page":"1152","article-title":"Uber rezidivierende Aphthose, durch ein Virus verursachte Geschwure am Mund, am Auge und an den Genitalien.","volume":"105","author":"Behc\u0327et","year":"1937","journal-title":"Dermatol Wochenschr."},{"key":"2021121002525309200_B2","first-page":"1572","article-title":"Vascular involvement in Behc\u0327et's disease.","volume":"19","author":"Koc","year":"1992","journal-title":"J Rheumatol."},{"key":"2021121002525309200_B3","first-page":"34","article-title":"Behc\u0327et's syndrome in children.","volume":"23","author":"Ozdagan","year":"1994","journal-title":"Rheumatology in Europe."},{"key":"2021121002525309200_B4","doi-asserted-by":"crossref","first-page":"878","DOI":"10.1001\/archinte.1986.00360170074012","article-title":"Thalidomide effects in Behc\u0327et's syndrome and pustular vasculitis.","volume":"146","author":"Jorizzo","year":"1986","journal-title":"Arch Intern Med."},{"key":"2021121002525309200_B5"},{"key":"2021121002525309200_B6"},{"key":"2021121002525309200_B7","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1111\/j.1442-200X.1997.tb03601.x","article-title":"Behc\u0327et's disease in children: a nationwide retrospective survey in Japan.","volume":"39","author":"Satoshi","year":"1997","journal-title":"Acta Paediatr Jpn."},{"key":"2021121002525309200_B8","first-page":"513","article-title":"Recognition of infantile-onset multisystem inflammatory disease as a unique entity.","volume":"130","author":"Hashkes","year":"1997","journal-title":"J Pediatr."},{"key":"2021121002525309200_B9","first-page":"1078","article-title":"Criteria for diagnosis of Behc\u0327et's disease.","volume":"335","author":"International Study Group for Behc\u0327et's Disease","year":"1990","journal-title":"Lancet."},{"key":"2021121002525309200_B10"},{"key":"2021121002525309200_B11","first-page":"393","article-title":"The emerging role of thalidomide therapy in HIV-infected patients.","volume":"14","author":"Haslett","year":"1997","journal-title":"Infect Med."},{"key":"2021121002525309200_B12","doi-asserted-by":"crossref","first-page":"1487","DOI":"10.1056\/NEJM199705223362103","article-title":"Thalidomide for the treatment of oral aphthous ulcers in patients with human immunodeficiency virus infection. National Institute of Allergy and Infectious Diseases AIDS Clinical Trials Group.","volume":"336","author":"Jacobson","year":"1997","journal-title":"N Engl J Med."},{"key":"2021121002525309200_B13","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/S0162-3109(96)00149-X","article-title":"Immunomodulatory assays to study structure-activity relationships of thalidomide.","volume":"35","author":"Shannon","year":"1997","journal-title":"Immunopharmacology."},{"key":"2021121002525309200_B14","doi-asserted-by":"crossref","first-page":"4082","DOI":"10.1073\/pnas.91.9.4082","article-title":"Thalidomide is an inhibitor of angiogenesis.","volume":"91","author":"D'Amato","year":"1994","journal-title":"Proc Natl Acad Sci U S A."},{"key":"2021121002525309200_B15","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/0024-3205(94)90099-X","article-title":"Thalidomide and the immune  response: simultaneous up- and down-regulation of different integrin receptors on human white blood cells.","volume":"55","author":"Nogueira","year":"1994","journal-title":"Life Sci."},{"key":"2021121002525309200_B16","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1097\/00007890-199111000-00029","article-title":"Immunosuppressive properties of thalidomide.","volume":"52","author":"Keenan","year":"1991","journal-title":"Transplantation."},{"key":"2021121002525309200_B17","first-page":"5","article-title":"Thalidomide's effectiveness in erythema nodosum leprosum is associated with a decrease in CD4+ cells in the peripheral blood.","volume":"63","author":"Shannon","year":"1992","journal-title":"Lepr Rev."},{"key":"2021121002525309200_B18","doi-asserted-by":"crossref","first-page":"443","DOI":"10.7326\/0003-4819-128-6-199803150-00004","article-title":"Thalidomide in the treatment of the mucocutaneous lesions of the Behc\u0327et syndrome. A randomized, double-blind, placebo-controlled trial.","volume":"128","author":"Hamuryudan","year":"1998","journal-title":"Ann Intern Med."},{"key":"2021121002525309200_B19","first-page":"26","article-title":"An internally controlled double-blind trial of thalidomide in severe erythema nodosum leprosum.","volume":"42","author":"Waters","year":"1971","journal-title":"Lepr Rev."},{"key":"2021121002525309200_B20","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1136\/ard.53.12.828","article-title":"Clinical experience with thalidomide in the management of severe oral and genital ulceration in conditions such as Behc\u0327et's disease: use of neurophysiological studies to detect thalidomide neuropathy.","volume":"53","author":"Gardner-Medwin","year":"1994","journal-title":"Ann Rheum Dis."},{"key":"2021121002525309200_B21","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1111\/j.1365-2133.1985.tb02323.x","article-title":"Development of polyneuropathy during thalidomide therapy.","volume":"112","author":"Wulff","year":"1985","journal-title":"Br J Dermatol."},{"key":"2021121002525309200_B22","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1177\/095646249100200514","article-title":"Thalidomide, peripheral neuropathy and AIDS.","volume":"2","author":"Fuller","year":"1991","journal-title":"Int J STD AIDS."}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/103\/6\/1295\/843941\/1295.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/103\/6\/1295\/843941\/1295.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T09:19:12Z","timestamp":1639127952000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/103\/6\/1295\/62321\/Thalidomide-Responsiveness-in-an-Infant-With-Behc"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,6,1]]},"references-count":22,"journal-issue":{"issue":"6","published-online":{"date-parts":[[1999,6,1]]},"published-print":{"date-parts":[[1999,6,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.103.6.1295","relation":{},"ISSN":["1098-4275","0031-4005"],"issn-type":[{"value":"1098-4275","type":"electronic"},{"value":"0031-4005","type":"print"}],"subject":[],"published":{"date-parts":[[1999,6,1]]}}}