PEDIATRICS Vol. 83 No. 6 June 1989, pp. 986-992
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Behcet Disease: Long-term Follow-up of Three Children and Review of the Literature

Yardena Rakover MD1, Hanna Adar MD1, Itamar Tal MD1, Yaron Lang MD1, and Amos Kedar MD1

1 The Departments of Pediatrics A and Ophthalmology, Central Emek Hospital, Afula, Israel

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.

Key Words: Behcet disease • aphthous stomatitis • genital ulcer • iritis

Submitted on December 28, 1987
Accepted on February 25, 1988




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