Behçet's disease : Report of twenty-five patients from the United States with prominent mucocutaneous involvement

• Published in: Journal of the American Academy of Dermatology 1996-05, Vol.34 (5), p.745-750
• Main Authors: MANGELSDORF, H. C, WHITE, W. L, JORIZZO, J. L
• Format: Article
• Language: English
• Subjects: Administration, Topical; Adolescent; Adult; Aged; Anti-Inflammatory Agents - therapeutic use; Behcet Syndrome - drug therapy; Behcet Syndrome - pathology; Biological and medical sciences; Colchicine - administration & dosage; Colchicine - therapeutic use; Dapsone - administration & dosage; Dapsone - therapeutic use; Dermatologic Agents - administration & dosage; Dermatologic Agents - therapeutic use; Female; Genital Diseases, Female - pathology; Genital Diseases, Male - pathology; Glucocorticoids - administration & dosage; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Injections, Intralesional; Male; Medical sciences; Methotrexate - administration & dosage; Methotrexate - therapeutic use; Middle Aged; Neutrophils - pathology; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Skin Ulcer - pathology; Stomatitis, Aphthous - pathology; Thalidomide - administration & dosage; Thalidomide - therapeutic use; United States; Vasculitis, Leukocytoclastic, Cutaneous - pathology
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/S0190-9622(96)90006-7

Behçet's disease is a multisystem disease that is rare in the United States. The purpose of our study was to assess the characteristics and treatment of a series of patients with Behçet's disease in the United States. A retrospective clinical review of 25 patients with Behçet's disease was performed, and histopathologic findings and therapeutic modalities were reviewed. All patients had oral and genital aphthae, and 22 of 25 patients had cutaneous lesions consistent with Behçet's disease. Eight of 25 patients had relatively severe systemic disease. Nine of 14 biopsy specimens showed a neutrophilic vascular reaction. Our therapeutic "ladder" included aggressive topical and intralesional corticosteroids, colchicine, dapsone, methotrexate, and thalidomide; we reserved systemic corticosteroids and immunosuppressive medications for severe ocular or severe systemic disease. This series of patients with Behçet's disease was characterized by patients with prominent mucocutaneous involvement and a low prevalence of ocular involvement. These findings may be attributed to patient selection from referral to a university dermatology clinic.