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报告1例多发性骨髓瘤伴角层下脓疱性皮肤病。患者男,63岁。因皮肤反复红斑、脓疱2年余,复发加重1个月就诊。皮损组织病理示角层下脓疱,其内可见大量的中性粒细胞,表皮棘层轻度肥厚,真皮小血管周围较多淋巴细胞浸润。皮损边缘组织直接免疫荧光示Ig A、Ig G、Ig M和C3均(-)。骨髓组织病理见较多浆细胞成片、灶性或散在分布。免疫组化示异形细胞CD138(+),浆细胞(plasma cell,PC)(+)、免疫球蛋白κ链(+)、免疫球蛋白λ链(-)。血清免疫固定电泳示一条M蛋白带,与抗Ig G和抗免疫球蛋白κ链形成特异性反应沉淀带。诊断:多发性骨髓瘤伴角层下脓疱性皮肤病。
Report 1 case of multiple myeloma with pustular skin disease. Male patient, 63 years old. Repeated erythema due to skin, pustules more than 2 years, recurrence increased 1 month treatment. Pathological lesions showed pustules under the horn, which can be seen a large number of neutrophils, epidermal spine mild hypertrophy, dermal small blood vessels around the more infiltration of lymphocytes. Direct immunofluorescence of marginal tissue showed IgA, IgG, IgM and C3 (-). Pathological bone marrow see more plasma cells into pieces, focal or scattered distribution. Immunohistochemistry showed that CD138 (+), plasma cell (PC) (+), immunoglobulin κ chain (+), immunoglobulin λ chain (-) Serum immunostaining showed a M protein band, forming specific reaction with anti-Ig G and anti-immunoglobulin κ chain. Diagnosis: Multiple myeloma with pustular skin disease under the horn.