Mucocutaneous manifestations of Behçet's disease.Acta Reumatol Port. 2013 Apr-Jun; 38(2):77-90.AR
Behçet's disease is a relapsing multisystem polysymptomatic disease with exacerbations and remissions defined by the presence of the major symptom, recurrent oral aphthous ulcers, plus two of the following: recurrent genital ulceration, eye lesions, skin lesions or a positive pathergy test. Mucocutaneous manifestations like oral and genital ulcers, and cutaneous lesions (papulopustular lesions, erythema nodosum-like lesions, cutaneous ulcers, superficial thrombophlebitis), are considered the \"fingerprint\" of the disease, being the most common and often the first signs to appear. Although the exact etiopathogenesis is still not known, genetic predispose and environmental factors may influence and contribute to the development of this disease. Diagnosis is based in the International Study Group criteria. During the last years, this disease has been largely studied and new immunological data and treatment strategies have been postulated. Despite that, further studies and attention to new data are needed.