华氏巨球蛋白血症所致大疱性皮肤病
Waldenström macroglobulinemia-induced bullous dermatosis.
作者信息
West N Y, Fitzpatrick J E, David-Bajar K M, Bennion S D
机构信息
Department of Dermatology, University of Colorado Health Sciences Center, Denver, USA.
出版信息
Arch Dermatol. 1998 Sep;134(9):1127-31. doi: 10.1001/archderm.134.9.1127.
BACKGROUND
Waldenström macroglobulinemia is a plasma cell dyscrasia of undetermined cause characterized by the monoclonal proliferation of lymphoplasmacytes in the bone marrow, lymph nodes, and spleen and elevated circulating levels and tissue deposition of monoclonal IgM produced by these aberrant cells. Rarely, cutaneous manifestations of this disease have been reported.
OBSERVATIONS
We report the case of a patient with bullous dermatosis induced by Waldenström macroglobulinemia and demonstrate the subepidermal location of the separation and the presence of IgM and kappa light chains by immunoperoxidase, immunofluorescent techniques, and electron microscopy with immunogold staining. Immunoblotting revealed a strong band at the 290-kd area.
CONCLUSIONS
The demonstration of the separation in the upper dermis at the site of IgM deposits suggests that these deposits may be an etiologic factor in this rare manifestation.
背景
华氏巨球蛋白血症是一种病因不明的浆细胞异常增生症，其特征为骨髓、淋巴结和脾脏中的淋巴浆细胞单克隆增殖，以及这些异常细胞产生的单克隆IgM循环水平升高和组织沉积。这种疾病的皮肤表现鲜有报道。
观察结果
我们报告了一例由华氏巨球蛋白血症引起的大疱性皮肤病患者，并通过免疫过氧化物酶、免疫荧光技术以及免疫金染色电子显微镜证实了表皮下分离的位置以及IgM和κ轻链的存在。免疫印迹显示在290-kd区域有一条强带。
结论
在IgM沉积部位的真皮上层显示出分离现象，这表明这些沉积物可能是这种罕见表现的病因。
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