Orozco-Covarrubias L, Guzmán-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R
J Eur Acad Dermatol Venereol. 2002 Jul;16(4):361-6. doi: 10.1046/j.1468-3083.2002.00442.x.
The aim was to be able to evaluate the diagnosis of two diseases by a consensus of clinical opinion used in the Department of Dermatology of the National Institute of Paediatrics in Mexico City. To differentiate between scleroderma 'en coup de sabre' (SCS) and progressive facial hemiatrophy (PFH), colour slides of 13 patients diagnosed as SCS and nine as PFH were examined by two dermatologists and microscopic slides by two pathologists. In both cases, the slides were randomly presented and no clinical information was given. The clinical and histopathological findings were statistically compared with two-tailed tests and alpha = 0.05. Kappa coefficients were obtained to evaluate the concordance between dermatologists, pathologists, and in terms of the consensus diagnosis. The usefulness of photographic assessment is limited by the inability to palpate the consistency of lesions. The most important clinical feature that differentiated both conditions was cutaneous sclerosis present in eight of 13 patients with SCS and in none of the PFH patients (P < 0.005). Other clinical features more frequently found in SCS were cutaneous hyperpigmentation and alopecia. The more frequent clinical features in PFH were total hemifacial involvement and ocular changes. Statistically significant histopathological features were: connective tissue fibrosis present in all cases with SCS and two of nine patients with PFH (P < 0.0002); adnexal atrophy present in 11 of 13 patients with SCS, and in three of nine with PFH (P < 0.02), and mononuclear cell infiltrates in all patients with SCS cf. six with PFH (P < 0.05). Our results suggest that in most cases it is possible to differentiate SCS from PFH based on clinicopathological findings.
目的是能够通过墨西哥城国家儿科研究所皮肤科采用的临床意见共识来评估两种疾病的诊断。为了区分“剑伤样”硬皮病（SCS）和进行性面部半侧萎缩（PFH），两名皮肤科医生检查了13例诊断为SCS的患者和9例诊断为PFH的患者的彩色幻灯片，两名病理学家检查了显微镜载玻片。在这两种情况下，幻灯片都是随机呈现的，且未提供临床信息。临床和组织病理学结果采用双尾检验进行统计学比较，α = 0.05。获得kappa系数以评估皮肤科医生、病理学家之间的一致性以及共识诊断的一致性。摄影评估的实用性受到无法触诊病变质地的限制。区分这两种情况的最重要临床特征是13例SCS患者中有8例出现皮肤硬化，而PFH患者中无一例出现（P < 0.005）。SCS中更常见的其他临床特征是皮肤色素沉着和脱发。PFH中更常见的临床特征是全面部受累和眼部改变。具有统计学意义的组织病理学特征为：所有SCS病例均存在结缔组织纤维化，9例PFH患者中有2例存在（P < 0.0002）；13例SCS患者中有11例出现附件萎缩，9例中有3例出现（P < 0.02），所有SCS患者均有单核细胞浸润，而PFH患者中有6例出现（P < 0.05）。我们的结果表明，在大多数情况下，根据临床病理结果可以区分SCS和PFH。
