familial Behcet-like autoinflammatory syndrome

An autoinflammatory disease that is characterized by characterized by ulceration of mucosal surfaces, particularly in the oral and genital areas and that has_material_basis_in heterozygous mutation in the TNFAIP3 gene on chromosome 6q23.

Signs and symptoms

• Oral ulcer
• Genital ulcers
• Polyarticular arthritis
• Skin rash
• Antinuclear antibody positivity
• Lupus anticoagulant
• Anterior uveitis
• Colitis
• Recurrent fever
• Decreased total lymphocyte count

Also known as: A20 haploinsufficiency