Behcet's disease in childhood

Abstract

Objective: to make Pediatricians aware of the fact that they must investigate Behcet's disease while performing differential diagnosis of recurrent aphthous stomatitis, even though it is a vasculitis of rare occurrence in early life. Methods: between June 1996 and December 2000, we retrospectively evaluated seven patients of our Pediatric Rheumatology Unit. Demographic, clinical, and laboratory data as well as data regarding treatment and follow-up were presented. Results: five out of seven patients were female (71.4%), four were non-Caucasian (57.1%), the mean age at onset was 8 years and 11 months (variation of 6 months to 13 years and 8 months), the mean period until diagnosis was 2 years and 3 months (variation of 2 months to 8 years) and the mean follow-up period was 4 years and 2 months (three patients without follow-up). The major criteria of diagnosis were: oral ulcers in seven patients (100%), genital ulcers in three patients (42.8%), ophthalmic alterations in four patients (57.1%) cutaneous vasculitis in one patient (14.2%) and positive pathergy test in one patient (14.2%). The minor criteria were: arthralgia/arthritis in five patients (71.4%), family history in two patients (28.5%), and sagittal sinus thrombosis in one patient (14.2%). The initial symptoms included recurrent aphthous stomatitis (more than three painful aphthous ulcers episodes in the period of 1 year), genital ulcers, arthralgia, fever and weight loss. The laboratory findings were: mild anemia in 1/6 patients, ESR>25 in 3/6 patients, increased serum gammaglobulin level in 2/4 patients, B5 histocompatibility antigen in 2/ 7 patients. The treatment included corticosteroids for 5/7 patients (4 oral, 1 intravenous and one local use), thalidomide for 4/7 patients, colchicine for 2/7 patients and dapsone for 1/7 patient. The outcome was favorable in 4/6, and 3/6 patients presented relapse. Conclusion: our results confirm the importance of considering the diagnosis of Behcet's disease in patients with recurrent oral and genital ulcers.

Recurrent Aphthous Stomatitis in Childhood and Adolescence: A Single‐Center Experience

“…Because oral aphthous ulcerations may be a symptom of other systemic diseases in children, a history or presence of genital ulcers is helpful in differentiating BD. Ophthalmic involvement is present in 28% to 68% of children with BD . Five percent of our patients had ocular disease related to BD.…”

“…Serum Ig levels are generally normal, although high and low serum IgA, IgG, IgD, and IgE levels have been reported in different groups of individuals with RAS . Hypergammaglobulinemia was found in 50% of the patients in a small group of children with BD . Oral ulceration is uncommon in selective IgA deficiency .…”

Behçet’s disease as cause of sinovenous thrombosis in a pediatric patient

“…The mean age at diagnosis is 10 years. Behçet's disease is not a familiar entity to most pediatricians due to its low incidence and limited recognition, since specific signs and symptoms sometimes appear at a later age [6]. Involvement of the CNS has been described in approximately 18% of patients, and is considered the most severe manifestation of the disease.…”

Juvenile Behсet's Disease: Clinical Observation

“…Подчеркивается, что при отсутствии внекишечных симптомов заболевания невозможно отли-чить интестинальную форму болезни Бехчета от болезни Крона [1]. К этому следует добавить, что диагностика болезни Бехчета основывается на совокупности клас-сификационных/диагностических признаков, развитие которых может быть растянутым на 5-6 и более лет [14,15]. О сложности верификации диагноза свидетель-ствует описание 17 вариантов диагностических/квали-фикационных критериев болезни Бехчета [16].…”

“…В биохимическом анализе сыворотки крови име-лась диспротеинемия: альбумин 48,98% (норма 53,0-68,0), альфа-1 глобулин -6,26% (норма 1,6-4,5), аль-фа-2 -23,01% (норма 5,7-11,5), бета -12,05% (норма 8,0-14,2), гамма -9,70% (норма 10, [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]5).…”