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首页> 外文期刊>The Journal of dermatology >Possible paraneoplastic syndrome case of bullous pemphigoid with immunoglobulin G anti-BP180 C-terminal domain antibodies associated with psoriasis and primary macroglobulinemia
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Possible paraneoplastic syndrome case of bullous pemphigoid with immunoglobulin G anti-BP180 C-terminal domain antibodies associated with psoriasis and primary macroglobulinemia

机译：牛皮癣和原发性大球蛋白血症相关的免疫球蛋白G抗BP180 C末端域抗体的大疱性类天疱疮的可能的副肿瘤综合征病例

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A 61-year-old Japanese man developed bullous skin lesions during topical therapy for psoriasis vulgaris. Physical examination demonstrated numerous tense bullae and scaly erythemas on the trunk and extremities. Histopathology of the skin biopsy demonstrated subepidermal bullae and lymphocytic infiltration with eosinophils in the dermis. Direct immunofluorescence revealed linear deposits of immunoglobulin (Ig)G, IgA and C3 along the basement membrane zone. Indirect immunofluorescence of 1 mol/L NaCl-split skin showed IgG reactivity with both epidermal and the dermal sides. IgM reactivity with both the epidermal and dermal sides was also detected. Enzyme-linked immunosorbent assays showed negative results for both BP180 and BP230. Immunoelectrophoresis of serum and bone marrow aspiration revealed underlying primary macroglobulinemia with M-proteinemia of IgM- type. Immunoblot analysis revealed IgG, but not IgM, antibodies to recombinant protein of BP180 C-terminal domain. We diagnosed the present case as bullous pemphigoid with IgG anti-BP180 C-terminal domain autoantibodies associated with primary macroglobulinemia and psoriasis vulgaris. Systemic administration of prednisolone 30 mg/day resulted in dramatic improvement of both bullous and psoriatic skin lesions. When the bullous and psoriatic lesions relapsed, DRC chemotherapy (dexamethasone, rituximab and cyclophosphamide) for macroglobulinemia was performed. Then, the psoriatic lesions improved and the bullous lesions disappeared. We suggested that the present case may be paraneoplastic syndrome of bullous pemphigoid associated with primary macroglobulinemia and psoriasis vulgaris.
机译：一名61岁的日本男子在寻常性牛皮癣的局部治疗期间出现大疱性皮肤损害。体格检查显示躯干和四肢上有大量紧张的大疱和鳞状红斑。皮肤活检的组织病理学表现为表皮下大疱和真皮中嗜酸性粒细胞浸润。直接免疫荧光显示免疫球蛋白（Ig）G，IgA和C3沿基底膜区线性沉积。 1 mol / L NaCl分离的皮肤的间接免疫荧光显示IgG与表皮和真皮侧都有反应。还检测到与表皮和真皮侧的IgM反应性。酶联免疫吸附试验对BP180和BP230均显示阴性结果。血清和骨髓穿刺的免疫电泳显示潜在的原发性巨球蛋白血症和IgM型M蛋白血症。免疫印迹分析揭示了针对BP180 C末端域重组蛋白的IgG，而非IgM。我们将本病例诊断为大疱性类天疱疮，伴有与原发性巨球蛋白血症和寻常型牛皮癣相关的IgG抗BP180 C末端结构域自身抗体。泼尼松龙30 mg /天的全身给药导致大疱性和银屑病性皮肤损伤的显着改善。当大疱性和银屑病性病变复发时，进行了针对大球蛋白血症的DRC化疗（地塞米松，利妥昔单抗和环磷酰胺）。然后，银屑病皮损得到改善，大疱性皮损消失。我们建议本病例可能是大疱性天疱疮伴副原发性巨球蛋白血症和寻常型牛皮癣的副肿瘤综合征。

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