Behçet's-Like Syndrome Associated With Psoriasis, Lichen Sclerosus and Variant in NF-κB1.

Behçet's disease is a rare multisystem inflammatory disorder characterised by recurrent oral and genital ulceration with systemic involvement. Heterozygous mutations in NF-κB1 have been associated with Behçet's-like presentations. We present a woman with psoriasis, lichen sclerosus, non-small-cell lung cancer, anogenital ulcers and features of a Behçet's-like syndrome associated with a novel NF-κB1 mutation. She responded well to adalimumab after failed treatment with systemic and topical corticosteroids, antibiotics and colchicine.

Abstract from PubMed