Escobosa Sánchez O María, Moreno Pascual P, Montes de Oca F, Milano Manso G, Jurado Ortiz A
Servicios de Pediatría, Hospital Materno-Infantil Carlos Haya, Málaga, Spain.
An Pediatr (Barc). 2004 Sep;61(3):266-9. doi: 10.1016/s1695-4033(04)78807-4.
Behcet's disease is a multisystemic vasculitis of unknown origin whose classical triad is oral and genital ulcers and uveitis. Deep venous thrombosis may sometimes be the first manifestation of this disease but is very rare in children. We report the case of a 14-year-old boy with deep venous thrombosis of the leg and a history of severe aphthous gingivostomatitis, perianal ulcers, Henoch-Schonlein purpura, ecchymotic lesions on the lower limbs and intermittent swelling of the left ankle. Complementary tests ruled out the main causes of thrombosis in children. HLA-B5 and B-51 study proved positive, which, together with the patient's history, suggested a diagnosis of Behcet's disease. This disease usually presents around the third decade of life and is very unusual in children. This case is exceptional because thrombosis as an early manifestation of Behcet's disease is very rare in children and because it led to the diagnosis of Behcet's disease in our patient.
白塞病是一种病因不明的多系统血管炎,其典型三联征为口腔和生殖器溃疡以及葡萄膜炎。深静脉血栓形成有时可能是该病的首发表现,但在儿童中非常罕见。我们报告了一例14岁男孩,患有腿部深静脉血栓形成,并有严重复发性口腔溃疡性龈口炎、肛周溃疡、过敏性紫癜、下肢瘀斑性病变以及左脚踝间歇性肿胀的病史。辅助检查排除了儿童血栓形成的主要原因。HLA - B5和B - 51检测呈阳性,结合患者病史,提示诊断为白塞病。这种疾病通常在生命的第三个十年左右出现,在儿童中非常少见。该病例很特殊,因为血栓形成作为白塞病的早期表现在儿童中非常罕见,且它使我们的患者得以诊断为白塞病。