Serra S, Ambrósio C, João Salvador M, Silva J, Serra D, Pedro Reis J, Malcata A
Serviço de Reumatologia, Hospitais da Universidade de Coimbra, Coimbra.
Acta Reumatol Port. 2010 Jan-Mar;35(1):66-71.
The authors present a clinical case of a 79 years old female patient, with a progressive cutaneous thickening of the face, trunk and limbs, lasting for 6 years. She also presented exertional dyspnea and intermittent solid dysphagia. The laboratory study identified IgG KAPA monoclonal protein and antinuclear antibodies with a speckled pattern. The nailfold capillaroscopy was normal. A detailed physical examination together with cutaneous histopathology led to the diagnosis of Scleromyxedema, a rare cutaneous scleroderma like disease. The differential diagnosis of cutaneous sclerosis is discussed.
作者介绍了一例79岁女性患者的临床病例，其面部、躯干和四肢皮肤进行性增厚，持续6年。她还出现劳力性呼吸困难和间歇性固体食物吞咽困难。实验室检查发现IgG KAPA单克隆蛋白和呈斑点状的抗核抗体。甲襞毛细血管镜检查正常。详细的体格检查及皮肤组织病理学检查确诊为硬化性黏液水肿，一种罕见的类似皮肤硬皮病的疾病。文中讨论了皮肤硬化的鉴别诊断。
