Rogowska Jagoda, Styrzyński Filip, Makowska Joanna, Brzezińska Olga
Department of Rheumatology, Medical University of Lodz, Łódź, Poland.
Rheumatol Int. 2025 Jan 8;45(1):22. doi: 10.1007/s00296-024-05775-2.
Scleromyxedema is a rare chronic fibromucinous disorder characterized by a generalized papular and sclerodermoid eruption. Despite its clinical significance, no definitive therapeutic guidelines exist for scleromyxedema, making management challenging. Herein, we present a case of a 76-year-old female patient referred for evaluation of systemic sclerosis, presenting with distinctive cutaneous manifestations and neurological symptoms. Investigations revealed monoclonal gammopathy (IgG Lambda subtype) and antinuclear antibodies, supporting a diagnosis of scleromyxedema. A multidisciplinary approach with intravenous immunoglobulin (IVIG) therapy resulted in significant improvement in neurological and cutaneous symptoms. This case underscores the challenges of diagnosing and managing scleromyxedema and highlights IVIG as a potential therapeutic option in the absence of standardized guidelines.
硬化性黏液水肿是一种罕见的慢性纤维黏液性疾病，其特征为全身性丘疹和硬皮病样皮疹。尽管具有临床意义，但目前尚无针对硬化性黏液水肿的确切治疗指南，这使得管理具有挑战性。在此，我们报告一例76岁女性患者，因系统性硬化症评估转诊而来，伴有独特的皮肤表现和神经症状。检查发现单克隆丙种球蛋白病（IgG λ亚型）和抗核抗体，支持硬化性黏液水肿的诊断。采用静脉注射免疫球蛋白（IVIG）治疗的多学科方法使神经和皮肤症状得到显著改善。该病例强调了硬化性黏液水肿诊断和管理的挑战，并突出了在缺乏标准化指南的情况下IVIG作为一种潜在治疗选择的作用。
