Incomplete Behcets’ Disease in a Teenager

Background: Behcets’ disease (BD) is a multisystemic vasculitis with various manifestations: mucocutaneous, articular, ocular, gastrointestinal, musculoskeletal, neurological, cardiac and pulmonary, the most common of which is the presence of painful ulcers lasting more than 14 days that occurs at east 3 times in a year, either alone or in combination with other lesions. Association with HLA-B51 has been described and sometimes have a positive pathergy test. Histopathologic evaluation reveals a neutrophilic vascular reaction or leukocytoclastic vasculitis. Clinical case: We report the case of a 14-year-old teenager with a past medical history of allergic rhinitis diagnosed, but with a 3-year history of painful oral ulcers that present more than 3 times per year; he presented oral ulcers, he did not present at the ocular and genital level. The biopsy samples of oral lesions did consistent with BD (showed narrowing of the vascular lumen due to neutrophil and lymphocyte infiltrate that completely affects the vascular wall) and HLA B 51 positive. Immunosuppressive treatment was started with mycophenolate 400 mg/ BSA for one year and 50 mg of prednisone OD followed by a gradual taper with good response to therapeutic approach and decrease of oral ulcers until they disappear. We present the case of a patient with an incomplete type of BD, with recurrent aphthous ulcers that present more than 3 times during a 12-month period as well as Indian descent, a biopsy with neutrophilic vascular reaction and positive HLA B51. BD is more likely to develop in children aged 11.7 to 14.5 years with diagnosis based on the presence of oral ulcers in 87 to 98% of the cases. Genital ulcers are far less common in children that in adults; BD is also more frequent among males. BD prognosis is dominated by ocular, neurological and vascular damage, with a poor functional and/or vital prognosis. Ocular involvement is severe and frequent, rapidly involving the visual prognosis. Treatment must be individualized according to the organ involved, good response has been described with intravenous steroids, which represent the mainstay of treatment, immunosuppressive the most commonly. Conclusions: Recurrent oral ulcers with more than three occurrences in one year must be considered as a sign to rule out BD. We must carry out an interrogation and when suspected, it must be confirmed with histopathological study and HLA B 51, regardless of the age of the patient. The diagnosis of BD is a challenge for the clinician.