AI Article Synopsis
- Behçet's disease is an autoimmune condition causing recurrent ulcers in the mouth and genitals, skin issues, and inflammation in the eyes, affecting multiple body systems.
- Genetic factors, particularly the HLA-B51 gene, may play a role in susceptibility, and the disease involves activation of neutrophils.
- Treatment varies based on severity, often involving immunosuppressive drugs, with serious long-term risks like blindness and dementia; a rare case in a child highlighted severe complications including ear canal necrosis and internal carotid artery rupture.
Article Abstract
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.
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| http://dx.doi.org/10.1016/j.ijporl.2003.12.016 | DOI Listing |
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