First Department of Critical Care, Evangelismos Hospital, National and Kapodistrian University of Athens, School of Medicine, University of Athens, Athens, Greece.
Dermatology Department, General Hospital of Athens, "Evangelismos", Athens, Greece.
Am J Med Sci. 2020 Aug;360(2):192-195. doi: 10.1016/j.amjms.2020.05.018. Epub 2020 May 20.
Dermato-neuro syndrome is a potentially fatal neurological complication of scleromyxedema consisting of fever, seizures, and coma. This is an overlooked scleromyxedema case of a 62-year-old female patient from 2-years ago. She was admitted to our ICU because of high fever, colloid speech, muscle ache, and nausea. Molecular methods in the cerebrospinal fluid for neurotropic viruses ruled out acute infectious encephalitis. Her thyroid hormones were within normal values while the serum protein electrophoresis confirmed the monoclonal gammopathy of immunoglobulin G lambda (IgG(λ)), known for the last 2 years. The subsequent bone-marrow biopsy excluded the development of multiple myeloma. The patient fulfilled fundamental diagnostic criteria of scleromyxedema (monoclonal gammopathy, normal thyroid function and the appearance of marked sclerosis and induration of the skin papules on the face, neck, extremities, and skin creases) presenting as dermato-neuro syndrome, which was histologically confirmed. She demonstrated a remarkable improvement after intravenous immunoglobulin treatment during the first 24 hours. Mimics of non-infectious acute encephalitis should include the clinical diagnosis of scleromyxedema, especially when patients present in the emergency department with acute fever, coma, and skin lesions of diffuse sclerodermoid and papular type.
皮肤神经综合征是一种潜在致命的神经并发症，由皮肤黏液水肿引起，表现为发热、癫痫发作和昏迷。这是一个 2 年前被忽视的皮肤神经综合征病例，患者为 62 岁女性。因高热、胶体言语、肌肉疼痛和恶心，她被收入我院 ICU。针对神经病毒的脑脊液分子方法排除了急性传染性脑炎。她的甲状腺激素值在正常范围内，而血清蛋白电泳证实了单克隆丙种球蛋白 IgG(λ)，这一情况在 2 年前就已经知晓。随后的骨髓活检排除了多发性骨髓瘤的发展。患者符合皮肤黏液水肿的基本诊断标准（单克隆丙种球蛋白病、甲状腺功能正常，以及面部、颈部、四肢和皮肤褶皱处皮肤丘疹出现明显硬化和硬结），表现为皮肤神经综合征，这一诊断已被组织学证实。在接受静脉注射免疫球蛋白治疗的前 24 小时内，她的病情显著改善。非传染性急性脑炎的类似症状应包括皮肤黏液水肿的临床诊断，特别是当患者因急性发热、昏迷和弥漫性硬皮样和丘疹样皮肤损伤出现在急诊时。
