Nickell Austin L, Corn Megan, Mannuru Devendranath, Hinze Alicia M
Internal Medicine, University of North Dakota School of Medicine and Health Sciences, Grand Forks, USA.
Obstetrics and Gynaecology, University of North Dakota School of Medicine and Health Sciences, Grand Forks, USA.
Cureus. 2023 Sep 9;15(9):e44968. doi: 10.7759/cureus.44968. eCollection 2023 Sep.
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.
意义未明的单克隆丙种球蛋白病(MGUS)背景下的2型硬化性黏液水肿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。其特征是皮肤慢性弥漫性硬结,始于上背部和颈部,由近端向远端发展,累及肩部、躯干和手臂;手部通常不受累。在此,我们报告一例长期硬化性黏液水肿进展累及手部和手指的罕见病例。该病例进一步并发了新发雷诺现象、脾肿大、淋巴结病、浆细胞瘤形成,并最终进展为多发性骨髓瘤。我们重点介绍了其复杂临床表现的鉴别诊断、已完成的检查以及当前的治疗选择。