Department of Dermatology, The Second Affiliated Hospital, Xi'an Jiaotong University, Shaanxi, 710049, People's Republic of China.
Arch Dermatol Res. 2024 Jun 6;316(6):333. doi: 10.1007/s00403-024-03090-1.
Stiff skin syndrome (SSS) is a rare disease characterized by thickened, indurated skin and limited joint movement. Multiple diverse phenotypes have been reported, and the correlation of severity with the clinical heterogeneity and histopathological findings of SSS needs to be refined.
To define subtypes based on clinical features and predict the prognosis of a new SSS classification.
Eighty-three patients with SSS were retrospectively reviewed for clinicopathological manifestations and routine laboratory workup, including 59 cases obtained from a PubMed search between 1971 and 2022 and 24 cases diagnosed in our department between 2003 and 2022.
Among the 83 patients, 27.7, 41, and 31.3% had classic widespread, generalized segmental, and localized SSS, respectively. Joint immobility was present in 100, 71, and 20% of classic, generalized, and localized cases, respectively. Histopathologic findings were common among the 3 groups, and based on that, we further found a difference in the distribution of proliferative collagen. 54.5% of classic and 50% of generalized cases occurred throughout the dermis or the subcutis, whereas 76% of localized cases were mainly involved in the reticular dermis or subcutis. In patients with incipient localized SSS, 42% (21/50) developed generalized SSS, and only 6% (3/50) progressed to classic SSS, whereas more than half of the incipient generalized SSS cases (60.6%, 20/33) developed classic SSS.
This retrospective study was limited to previously published cases with limited data.
We propose a distinct clinical classification characterized by lesion distribution, including classic widespread, generalized segmental, and localized SSS, associated with disease severity and prognosis.
硬皮病综合征（SSS）是一种罕见疾病，其特征为皮肤增厚、硬化和关节运动受限。已有多种不同表型的报道，需要对 SSS 严重程度与临床异质性和组织病理学表现的相关性进行精细化研究。
基于临床特征定义亚型，并预测新的 SSS 分类的预后。
回顾性分析 83 例 SSS 患者的临床病理表现和常规实验室检查结果，其中 59 例来自 1971 年至 2022 年期间的 PubMed 检索，24 例来自 2003 年至 2022 年期间我院的诊断病例。
83 例患者中，27.7%、41%和 31.3%分别为经典弥漫型、广泛节段型和局限性 SSS。100%、71%和 20%的经典弥漫型、广泛节段型和局限性病例存在关节活动受限。3 组患者的组织病理学表现均较常见，在此基础上，我们进一步发现增生性胶原的分布存在差异。54.5%的经典弥漫型和 50%的广泛节段型病例发生于真皮或皮下组织，而 76%的局限性病例主要累及网状真皮或皮下组织。在初发局限性 SSS 患者中，42%（21/50）进展为广泛节段型 SSS，仅有 6%（3/50）进展为经典弥漫型 SSS，而初发广泛节段型 SSS 患者中超过一半（60.6%，20/33）进展为经典弥漫型 SSS。
本回顾性研究仅限于已发表的病例，数据有限。
我们提出了一种明确的临床分类，其特征为病变分布，包括经典弥漫型、广泛节段型和局限性 SSS，与疾病严重程度和预后相关。
