Kövary P M, Vakilzadeh F, Macher E, Zaun H, Merk H, Goerz G
Arch Dermatol. 1981 Sep;117(9):536-9. doi: 10.1001/archderm.1981.01650090018016.
A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.
在3例患有长期广泛性硬化性黏液水肿(布施克病)的患者中观察到单克隆丙种球蛋白病。所有患者均无多发性骨髓瘤的证据。直接免疫荧光显微镜检查未检测到皮肤中单克隆免疫球蛋白的沉积。与硬化性黏液水肿(黏液性苔藓)不同,硬化性黏液水肿在临床和组织学上可与之区分,2例患者的单克隆免疫球蛋白为IgG2-κ和IgG3-κ型。3例患者中只有1例有IgG1-λ副蛋白血症,这在硬化性黏液水肿中很常见。我们的研究结果表明,弥漫性硬化性黏液水肿可能以副蛋白血症为特征,但单克隆免疫球蛋白在该疾病发病机制中的可能作用尚未明确。