Abstract：
BACKGROUND: Stiff skin syndrome (SSS) is a rare disease characterized by sclerosis of the skin. Cases of both widespread and segmental SSS have been reported.
OBJECTIVE: To report the clinical and histopathological characteristics of a large series of SSS.
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.
RESULTS: 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.
CONCLUSIONS: Segmental SSS represents the major variant of SSS. Histopathologically, SSS shows proliferation of fibroblasts, sclerosis and an absence of inflammation.
OBJECTIVE: To report the clinical and histopathological characteristics of a large series of SSS.
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.
RESULTS: 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.
CONCLUSIONS: Segmental SSS represents the major variant of SSS. Histopathologically, SSS shows proliferation of fibroblasts, sclerosis and an absence of inflammation.
摘要：
背景：皮肤僵硬综合征（SSS）是一种罕见的疾病，其特征是皮肤硬化。已经报道了广泛和节段性SSS的病例。
目的：报告一系列SSS的临床和组织病理学特征。
方法：我们回顾性分析了从皮肤科收集的广泛和节段性SSS的临床和组织病理学特征。我们还比较了节段性SSS和硬皮之间的组织病理学。
结果：31例，包括三个广泛的SSS和28个分段SSS，被收集。广泛的SSS的皮肤病变通常表现为集中在腰部的皮肤硬化，臀部,大腿,四肢近端,和肩膀有特定的异常步态和姿势。节段性SSS的皮肤病变通常表现为累及大腿的硬化斑块，腰部和臀部，与多毛症有关，色素沉着和鹅卵石外观。节段性SSS通常不会引起关节受限或严重的身体不适。组织病理学,SSS显示真皮或皮下组织中的成纤维细胞增殖和胶原硬化。与硬皮相比，SSS显示出更明显的成纤维细胞增殖，并且完全没有淋巴细胞浸润。
结论：分段SSS代表SSS的主要变体。组织病理学,SSS显示成纤维细胞的增殖，硬化和没有炎症。
目的：报告一系列SSS的临床和组织病理学特征。
方法：我们回顾性分析了从皮肤科收集的广泛和节段性SSS的临床和组织病理学特征。我们还比较了节段性SSS和硬皮之间的组织病理学。
结果：31例，包括三个广泛的SSS和28个分段SSS，被收集。广泛的SSS的皮肤病变通常表现为集中在腰部的皮肤硬化，臀部,大腿,四肢近端,和肩膀有特定的异常步态和姿势。节段性SSS的皮肤病变通常表现为累及大腿的硬化斑块，腰部和臀部，与多毛症有关，色素沉着和鹅卵石外观。节段性SSS通常不会引起关节受限或严重的身体不适。组织病理学,SSS显示真皮或皮下组织中的成纤维细胞增殖和胶原硬化。与硬皮相比，SSS显示出更明显的成纤维细胞增殖，并且完全没有淋巴细胞浸润。
结论：分段SSS代表SSS的主要变体。组织病理学,SSS显示成纤维细胞的增殖，硬化和没有炎症。
