Clinical features and differential diagnosis in pediatric Behçet’s Disease

Abstract

Behçet’s disease (BD) is a vasculitis characterized by recurrent attacks and multiple manifestations, including oral and genital ulcers, ocular, cutaneous, gastrointestinal, neurological, articular, and vascular findings. The disease affects any type and size of vessel, and the etiology remains unclear. The disease mainly begins in young adulthood and is rarely seen in childhood. Pediatric BD is defined as a fully manifested disease up to the age of 16 years and consists of 2.5–14% of all cases. Majority of studies indicate that there may be some differences between pediatric BD and adult BD: Family history is higher in frequency in pediatric BD than in adult BD. The most common manifestations are mucocutaneous lesions, with oral ulcers being the most common initial presentation. While gastrointestinal symptoms, central nervous system involvement, and arthralgia are more often seen in children, genital ulcers are more common in adults. Furthermore, the current literature indicates that pediatric BD has a relatively mild course, with a low severity score and activity index. The diagnosis of pediatric BD is difficult and differential diagnosis includes many diseases particularly autoinflammatory syndromes such as A20 haploinsufficiency, periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, cryopyrin-associated periodic syndrome, tumor necrosis factor receptor-associated periodic syndrome and deficiency of adenosine deaminase-2 and neutrophilic dermatoses such as pyoderma gangrenosum and Sweet’s syndrome, inflammatory bowel diseases, and other vasculitides.