Department of Dermatology, Xijing Hospital, the Fourth Military Medical University, No.127 of Changlexi Road, Xian 710032, China, Department of Dermatology, Zigong Fourth People's Hospital, Zigong, Sichuan, China.
Department of Dermatology, Xijing Hospital, the Fourth Military Medical University, No.127 of Changlexi Road, Xian 710032, China.
Eur J Dermatol. 2023 Jun 1;33(3):235-240. doi: 10.1684/ejd.2023.4477.
Stiff skin syndrome (SSS) is a rare disease characterized by sclerosis of the skin. Cases of both widespread and segmental SSS have been reported.
To report the clinical and histopathological characteristics of a large series of SSS.
MATERIALS & METHODS: We retrospectively analysed the clinical and histopathological characteristics of widespread and segmental SSS collected from a dermatology department. We also compared histopathology between segmental SSS and morphea.
Thirty-one cases, including three widespread SSS and 28 segmental SSS, were collected. Skin lesions of widespread SSS generally showed skin sclerosis concentrating over the lumbar, buttocks, thighs, proximal part of limbs, and shoulders with specific abnormal gait and posture. Skin lesions of segmental SSS generally showed sclerotic plaques involving the thigh, lumbar area and buttocks, associated with hypertrichosis, hyperpigmentation and a cobblestone appearance. Segmental SSS did not typically cause joint limitation or serious physical discomfort. Histopathologically, SSS showed proliferation of fibroblasts and sclerosis of collagen in the dermis or subcutaneous tissue. Compared with morphea, SSS showed more prominent proliferation of fibroblasts and completely lacked lymphocyte infiltration.
Segmental SSS represents the major variant of SSS. Histopathologically, SSS shows proliferation of fibroblasts, sclerosis and an absence of inflammation.
硬皮病综合征（SSS）是一种以皮肤硬化为特征的罕见疾病。广泛型和节段型 SSS 均有报道。
报告一系列广泛型和节段型 SSS 的临床和组织病理学特征。
我们回顾性分析了皮肤科收集的广泛型和节段型 SSS 的临床和组织病理学特征。我们还比较了节段型 SSS 和硬斑病的组织病理学表现。
共收集到 31 例患者，包括 3 例广泛型 SSS 和 28 例节段型 SSS。广泛型 SSS 的皮肤病变通常表现为集中于腰部、臀部、大腿、四肢近端和肩部的皮肤硬化，伴有特定的异常步态和姿势。节段型 SSS 的皮肤病变通常表现为累及大腿、腰部和臀部的硬化斑块，伴有多毛症、色素沉着和鹅卵石样外观。节段型 SSS 通常不会导致关节受限或严重的身体不适。组织病理学上，SSS 表现为真皮或皮下组织成纤维细胞增殖和胶原硬化。与硬斑病相比，SSS 显示出更明显的成纤维细胞增殖，完全缺乏淋巴细胞浸润。
节段型 SSS 是 SSS 的主要变异型。组织病理学上，SSS 表现为成纤维细胞增殖、硬化和无炎症。
