Anti‐BP230‐type papular non‐bullous pemphigoid with granular immunoglobulin G deposition at the papillary dermis
Anti‐BP230‐type papular non‐bullous pemphigoid with granular immunoglobulin G deposition at the papillary dermis
复制标题
抗 BP230 型丘疹性非大疱性类天疱疮，真皮乳头层有颗粒状免疫球蛋白 G 沉积
DOI：
10.1111/1346-8138.16070
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发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Ishii Norito
中科院分区：
文献类型：
--
作者：
Dainichi Teruki;Kaku Yo;Izumi Maiko;Kataoka Koki;Morishita Yoshiko;Koga Hiroshi;Ishii Norito
约5%的大疱性类天疱疮（BP）患者的血清仅与BP 230反应。1抗BP 230型BP通常表现出较低的严重程度，并可能具有局灶性受累。2抗BP 230 IgG抗体在BP中的致病性参与仍然存在争议3，4，因为BP 230是一种细胞内斑蛋白。我们报告一例抗BP 230型BP表现出全身性丘疹和斑块与颗粒状IgG沉积在乳头状真皮。患者女，50多岁，全身出现泛发性湿疹样丘疹和斑块。她三年前起了水泡。皮肤活检显示表皮下水疱和C3沉积在基底膜区（BMZ）。BP 180 IgG自身抗体的血清滴度为170 U/mL。患者被诊断为BP，并接受泼尼松龙（40 mg/天）治疗。水疱消失，血清BP 180 IgG抗体转阴。逐渐减少全身皮质类固醇;然而，逐渐地，红色丘疹复发。这些丘疹用甲泼尼龙24 mg、咪唑立宾150 mg、二苯砜75 mg每日一次和局部二氟拉松二醋酸酯治疗。检查时，躯干和四肢散在红色至棕色、有光泽的平顶丘疹和斑块（图1a）。实验室检查显示血白细胞11，210/mm 3，淋巴细胞620/mm 3，嗜酸性粒细胞170/mm 3，0.03 mg/dL
Around 5% of the bullous pemphigoid (BP) patients’ sera react with BP230 only. 1 Anti-BP230-type BP generally show lower severity, and possibly with focal involvement. 2 The pathogenic involvement of anti-BP230 IgG antibodies in BP remains controversial3, 4 because BP230 is an intracellular plakin protein. We report a case of anti-BP230-type BP that exhibited generalized pruritic papules and plaques with granular IgG deposition in the papillary dermis. A woman in her 50s presented with generalized pruritic erythematous papules and plaques over her body. She had developed blisters three years prior. Skin biopsy revealed subepidermal blistering and C3 deposition in the basement membrane zone (BMZ). The serum titer of the BP180 IgG autoantibodies was 170 U/mL. The patient was diagnosed with BP and treated with prednisolone (40 mg/day). The blisters disappeared, and serum BP180 IgG antibodies became negative. Systemic corticosteroids were tapered off; however, gradually, the reddish papules recurred. These papules were treated with methylprednisolone 24 mg, mizoribine 150 mg, diaphenylsulfone 75 mg daily, and topical diflorasone diacetate. On examination, red-to-brown, shiny, flat-topped papules and plaques were scattered on the trunk and extremities (Figure 1a). Laboratory examination revealed 11,210/mm3 blood leukocytes, 620/mm3 lymphocytes, 170/mm3 eosinophils, 0.03 mg/dL