Mangelsdorf H C, White W L, Jorizzo J L
Department of Dermatology, Bowman Gray School of Medicine, Winston-Salem, NC 27157-1071, USA.
J Am Acad Dermatol. 1996 May;34(5 Pt 1):745-50. 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.
白塞病是一种多系统疾病，在美国较为罕见。
我们研究的目的是评估美国一系列白塞病患者的特征及治疗情况。
对25例白塞病患者进行回顾性临床分析，并对组织病理学结果及治疗方式进行评估。
所有患者均有口腔和生殖器溃疡，25例患者中有22例有符合白塞病的皮肤损害。25例患者中有8例患有相对严重的系统性疾病。14份活检标本中有9份显示中性粒细胞血管反应。我们的治疗“阶梯”包括积极的局部和病灶内皮质类固醇、秋水仙碱、氨苯砜、甲氨蝶呤和沙利度胺；对于严重眼部或严重系统性疾病，我们保留全身皮质类固醇和免疫抑制药物。
这一系列白塞病患者的特点是黏膜皮肤受累明显，眼部受累患病率低。这些发现可能归因于从转诊至大学皮肤科诊所挑选的患者。
