硬化性水肿(Arndt-Gottron综合征)1例报告

D. Popović, M. Paravina, D. Jovanovic, Vesna Karanikolić, D. Ljubisavljević
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引用次数: 2

摘要

黏液水肿地衣,又称丘疹性黏液病,是一种原发性弥漫性皮肤黏液病。这是一种罕见的皮肤黏液性水肿,其特征是形成大量的地衣样丘疹。硬黏液性水肿,也被称为Arndt-Gottron综合征,是一种罕见的、汇合性、丘疹性和硬化性的黏液性地衣水肿,其特征是丘疹下皮肤弥漫性增厚。这种情况与全身甚至致命的表现、成纤维细胞增殖和透明质酸的酸性粘多糖的积累有关。血清IgG类副蛋白血症总是存在的,它可以在所有患者中检测到,如果适当,甚至使用重复检测。在此,我们提出一位67岁的患者,有2年的皮肤问题史。除了高血压和糖尿病,他没有其他健康问题,这两种疾病都是在15年前被诊断出来的。检查时,患者表现为硬皮样病变,暴露皮肤弥漫性假性硬皮增厚,小口和硬指样改变;在面部,有大量实心的、有光泽的、直径2 - 4毫米的皮肤颜色的地衣样丘疹,分布在前额、眉间区、鼻唇沟、口周区、耳垂和颈部。组织病理学检查:真皮中部有明显的胶原和纤维化,成纤维细胞增多;胶原蛋白束排列不规则、断裂;阿利新蓝阳性沉积物,外观与酸性粘蛋白一致。血清蛋白电泳检测IgG副蛋白血症。患者接受系统皮质类固醇治疗9个月,随后引入甲氨蝶呤,结果令人满意。硬化黏液水肿的病因尚不清楚,因为纯化的IgG副蛋白本身对成纤维细胞增殖没有直接影响。在硬黏液性水肿中,提出了许多治疗方法,但不幸的是效果有限。总之,我们报告一例成年男性与地衣样丘疹;经过两年的进展,他们发展为硬化性水肿,并对常规治疗表现出良好的反应。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Scleromyxedema (Arndt-Gottron Syndrome): a Case Report
Abstract Lichen myxedematosus, also known as papular mucinosis, is a primary diffuse cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, also known as Arndt–Gottron syndrome, is a rare, confluent, papular and sclerotic variant of lichen myxedematosus, characterized by diffuse thickening of the skin underlying the papules. The condition is associated with systemic, even lethal manifestations, fibroblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients if appropriate or even repeat testing is used. Herein, we present a 67-year-old patient with a 2-year history of skin problems. He had no health problems other than hypertension and diabetes, wich were both diagnosed 15 years before. On examination, the patient exhibited sclerodermoid lesions with diffuse pseudo-sclerodermatous thickening of the exposed skin, microstomia and sclerodactyly-like changes; on the face, there were numerous solid, shiny 2 - 4 mm in diameter skin-coloured lichenoid papules, scattered across the forehead, glabellar area, nasolabial folds, perioral region, ear lobes and the neck. Histopathological examination revealed: highly distinctive collagenosis and fibrosis in the middle dermis, increased fibroblasts; collagen bundles with irregular arrangement and fragmentation; alcian blue-positive deposits with appearance consistent with acid mucins. Serum protein electrophoresis detected IgG lambda paraproteinemia. The patient was treated with systematic corticosteroids during 9 months with subsequent introduction of methotrexate and showed satisfactory results. The etiology of scleromyxedema remains unknown, since the purified IgG paraprotein itself has no direct effects on fibroblast proliferation. In scleromyxedema, numerous therapeutic modalities are proposed, unfortunatelly with limited effects. In colclusion, we report a case of an adult male with lichenoid papules; after a two-year progression, they evolved into scleromyxedema and exhibited well response to conventional therapy.
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期刊介绍: Serbian Journal of Dermatology and Venereology is a journal of the Serbian Association of Dermatologists and Venereologists. The journal is published in English, quarterly and intended to provide rapid publication of papers in the field of dermatology and venereology. Manuscripts are welcome from all countries in the following categories: editorials, original studies, review articles, professional articles, case reports, and history of medicine.
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