Vernal Sebastian, Julio Tamiris, Cruz Fernanda, Turatti Aline, Ishii Norito, Hashimoto Takashi, Roselino Ana Maria
Ana Maria Roselino, MD, PhD, University Hospital, Ribeirão Preto Medical School, University of São Paulo, Av. Bandeirantes 3900, Ribeirão Preto, SP, Brazil 14049-900;
Acta Dermatovenerol Croat. 2018 Jun;26(2):179-182.
Bullous pemphigoid (BP) has been associated with neurological disorders (NDs), which has led to the hypothesis that molecular mimicry exists between hemidesmosomal proteins and neuronal peptides. A 79-year-old hemiparetic woman presented with tense bullae affecting exclusively her right paretic upper limb for three months. Histopathology, taken from the perilesional area, revealed an inflammatory infiltrate with predominant eosinophils. IIF evidenced linear IgG deposition in the epidermal side of the cleavage. ELISA detected circulating anti-BP180 and anti-BP230 autoantibodies. Immunoblotting exhibited unspecific reactivity against the 190-kDa periplakin in normal human epidermal extract. The immunocompromised cutaneous district concept may explain the possible mechanism for the exclusive involvement of the autoimmune blistering disease in lymphedematous hemiparetic upper limb.
大疱性类天疱疮(BP)与神经疾病(NDs)有关,这导致了一种假说,即半桥粒蛋白和神经肽之间存在分子模拟。一名79岁的偏瘫女性出现紧张性大疱,仅累及她右侧偏瘫的上肢,持续了三个月。取自病损周围区域的组织病理学检查显示有以嗜酸性粒细胞为主的炎性浸润。间接免疫荧光法(IIF)证明在裂隙的表皮侧有线性IgG沉积。酶联免疫吸附测定(ELISA)检测到循环抗BP180和抗BP230自身抗体。免疫印迹显示在正常人表皮提取物中对190-kDa周膜蛋白有非特异性反应。免疫受损皮肤区域的概念可能解释了自身免疫性水疱病仅累及淋巴水肿性偏瘫上肢的可能机制。