Jorizzo J L, Abernethy J L, White W L, Mangelsdorf H C, Zouboulis C C, Sarica R, Gaffney K, Mat C, Yazici H, al Ialaan A
Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, NC 27157-1071, USA.
J Am Acad Dermatol. 1995 Jun;32(6):968-76. doi: 10.1016/0190-9622(95)91333-5.
Although four of five of the new international criteria for the diagnosis of Behçet's disease relate to mucocutaneous lesions, disagreement exists as to the exact nature of cutaneous lesions (e.g., vessel-based vs follicular).
Our purpose was to review clinical data, clinical photographs, and skin biopsy specimens from multiple medical centers throughout the world to monitor current practice in the implementation of mucocutaneous diagnostic criteria for Behçet's disease.
Ten medical centers responded to a request to collaborate by sending clinical data, photographs of cutaneous lesions, and biopsy specimens from 22 patients.
Of specimens from 22 patients, 14 revealed a histopathologic pattern of neutrophils containing perivascular and interstitial inflammation, whereas specimens from three patients revealed only mononuclear cells in a vessel-based pattern. Biopsy specimens from three patients revealed primarily folliculocentric inflammation and an additional two specimens were from erythema nodosum-like lesions.
Perivascular inflammation was the predominant histopathologic finding in specimens of cutaneous lesions in this clinical series. Folliculocentric lesions could not be predicted on the basis of review of clinical photographs. Histopathologic assessment of cutaneous lesions is crucial if the proposal is accepted that exclusion of folliculocentric lesions is important to ensure accurate implementation of diagnostic criteria in patients with suspected Behçet's disease.
尽管白塞病新的国际诊断标准中有五项中的四项与黏膜皮肤病变有关，但对于皮肤病变的确切性质（例如，基于血管的病变与毛囊性病变）仍存在分歧。
我们的目的是回顾来自世界各地多个医疗中心的临床数据、临床照片和皮肤活检标本，以监测白塞病黏膜皮肤诊断标准实施中的当前实践情况。
十个医疗中心响应了合作请求，提供了22例患者的临床数据、皮肤病变照片和活检标本。
在22例患者的标本中，14例显示出含有血管周围和间质炎症的中性粒细胞的组织病理学模式，而3例患者的标本仅显示出基于血管模式的单核细胞。3例患者的活检标本主要显示以毛囊为中心的炎症，另外2例标本来自结节性红斑样病变。
在这个临床系列中，血管周围炎症是皮肤病变标本中主要的组织病理学发现。基于临床照片的审查无法预测以毛囊为中心的病变。如果接受排除以毛囊为中心的病变对于确保疑似白塞病患者诊断标准的准确实施很重要这一建议，那么皮肤病变的组织病理学评估至关重要。
