Skin Manifestations of Behçet’s Disease

Abstract

Behçet’s disease (BD) is a systemic vasculitis of unknown etiology involving the small and large vessels of both the venous and arterial systems. This disease, first recognized to be a new entity by Turkish dermatologist Hulusi Behçet, was termed for triple symptom complex of oral aphthae, genital ulcers, and hypopyon uveitis until BD became the accepted designation for this disorder. While features constituting the “triple symptom complex” are most commonly encountered among patients with BD, subsequent observations showed that this entity is a multisystem disease characterized by variable clinical manifestations. Mucocutaneous features are the most common and the presenting symptoms of the disease, while eye, vascular, and neurological elements are the most serious. Although mucocutaneous symptoms are not life threatening, they are associated with a substantial decrease in quality of life of BD patients. Skin lesions other than oral and genital ulcers are seen in approximately 80 % of patients with BD. These lesions can be divided into three broad categories: (1) nodular lesions (erythema nodosum-like lesions and superficial thrombophlebitis), (2) papulopustular and acneiform lesions, and (3) other lesions such as skin ulcers and Sweet’s syndrome. In this chapter, important features of mucocutaneous lesion as well as their differential diagnoses were discussed.