Clerici T, Faure M, Chouvet B, Perrot H, Hermier C
Dermatologica. 1983;166(5):240-6.
A monoclonal gammopathy was observed in 3 adult patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. The monoclonal immunoglobulin was in every case of the IgG-kappa type. Bence Jones proteinuria was noted in 1 case (kappa). Deposition of monoclonal IgG-kappa in the skin was not detected by immunofluorescence microscopy. These findings and previous reports of 5 cases suggest that diffuse scleredema may be frequently associated with paraproteinemia, but the role of monoclonal immunoglobulins in the pathogenesis of the disease remains to be clarified.
在3例患有长期广泛硬肿病(布施克病)的成年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。单克隆免疫球蛋白在每种情况下均为IgG-κ型。1例患者出现本周蛋白尿(κ型)。免疫荧光显微镜检查未检测到皮肤中单克隆IgG-κ的沉积。这些发现以及之前5例病例的报告表明,弥漫性硬肿病可能经常与副蛋白血症相关,但单克隆免疫球蛋白在该疾病发病机制中的作用仍有待阐明。