Behçet's disease: external ear involvement

Behçet's disease is an autoimmune vasculitis characterized by recurrent episodes of oral and genital ulcerations, skin lesions and uveitis. It also affects neurological, vascular, articular and gastrointestinal systems. Although etiology and pathogenesis are under investigation, studies have shown that HLA-B51 influences genetic susceptibility. The development of the disease is associated to neutrophil activation. Treatment depends on the severity of the disease and immunosuppressive drugs are often used. Blindness and dementia are long-term concerns. We report an unprecedented case of a child with Behçet's disease presenting a skin lesion with necrosis of the external ear canal, facial paresis, Horner's syndrome and rupture of the internal carotid artery.