PDF(Pubmed)
Abstract：
Type 2 scleredema on the background of monoclonal gammopathy of undetermined significance (MGUS) is a rare and progressive connective tissue disorder with very few cases reported to date. It is characterized by chronic and diffuse induration of the skin that begins in the upper back and neck and progresses proximally to distally, involving the shoulders, trunk, and arms; the hands are usually spared. Here, we present an unusual case of long-standing scleredema that progressed to involve the hands and fingers. This case was further complicated by new-onset Raynaud\'s phenomenon, splenomegaly, lymphadenopathy, the development of a plasmacytoma, and eventual progression to multiple myeloma. We highlight the differential diagnoses for his complex presentation, the workup that was completed, and current treatment options.
摘要：
以意义不明的单克隆丙种球蛋白病（MGUS）为背景的2型硬肿症是一种罕见的进行性结缔组织疾病，迄今为止报道的病例很少。它的特征是皮肤的慢性和弥漫性硬化，始于上背部和颈部，并向近端进展到远端，涉及到肩膀，树干,和手臂；手通常可以幸免。这里,我们提出了一个不寻常的情况下，长期进展到涉及手和手指。这个病例因新出现的雷诺现象而进一步复杂化，脾肿大,淋巴结病，浆细胞瘤的发展，最终进展为多发性骨髓瘤。我们强调他复杂的表现的鉴别诊断，完成的工作，和目前的治疗选择。
