Behçet's Disease

This is a multisystem disorder characterized by oral and genital aphthae (Figure A). Other symptoms include a myriad of cutaneous findings; variable systemic features include uveitis, synovitis, meningoencephalitis, and large- and smaller-vessel vascular disease.⁵

The cutaneous lesions of Behet's disease (BD) include erythema nodosum–like nodules (Figure B), vesicles, pustules, Sweet's syndrome, a pustular pathergic tissue reaction to needle trauma, superficial migratory thrombophlebitis, ulceration, pyoderma gangrenosum–like lesions, infiltrative erythema, and acneiform folliculitis.⁵ It is important to recognize these manifestations because in most patients they precede potentially life-threatening events, such as arterial and venous occlusions, aneurysms, and brain stem dysfunction, as well as gastrointestinal ulceration, meningoencephalitis, and blindness.

In 1992, the International Study Group for Behet's Disease set forth criteria for the diagnosis of BD.⁶ These include the presence of oral ulcerations plus two of the following symptoms: genital ulcerations, skin lesions, and eye involvement. Although the cause of BD is unknown, some researchers believe it may represent an abnormal immune reaction to infectious agents.⁵

Management options include corticosteroids and other immunosuppressives, such as azathioprine and cyclophosphamide. Other agents-such as colchicine, dapsone, cyclosporine, and thalidomide-have been used to inhibit neutrophil chemotaxis.

References:

REFERENCES:1. Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am. 1995;21:1097-1113.
2. Schneider JW, Jordaan HF, Geiger DH, et al. Erythema induratum of Bazin: a clinicopathologic study of 20 cases and detection of Mycobacterium tuberculosis DNA in skin lesions by polymerase chain reaction. Am J Dermatopathol. 1995;17:350-356.
3. Daoud MS, Gibson LE, DeRemee RA, et al. Cutaneous Wegener's granulomatosis: clinical, histopathologic, and immunopathologic features of thirty patients. J Am Acad Dermatol. 1994;31:605-612.
4. Chow RK, Benny WB, Coupe RL, et al. Erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with intermittent plasma exchange. Arch Dermatol. 1996;132:1360-1364.
5. Magro CM, Crowson AN. Cutaneous manifestations of Behçet's disease. Int J Dermatol. 1995;14:159-165.
6. International Study Group for Behçet's Disease. Evaluation of diagnostic ('classification') criteria in Behçet's disease-towards internationally agreed criteria. Br J Dermatol. 1992;31:299-308.