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Abstract：
Pemphigus is a rare disease characterized by bullous lesions of the skin and mucous membranes. The aetiology is autoimmune and related to the formation of IgG autoantibodies against desmogleins, which are structural proteins of desmosomes that ensure the stability of contacts between cells. Cardiac involvement in patients with pemphigus is poorly documented. We report the data in the literature on this topic and a case of pemphigus-associated autoimmune myocarditis with damage of intercalated disc responding to immunosuppressive therapy. The occurrence of cardiomyopathy with left ventricular dysfunction in patients affected by pemphigus should be appropriately screened with endomyocardial biopsy as it could be the myocardial extension of a potentially reversible autoimmune disorder.
摘要：
天疱疮是一种罕见的疾病，其特征是皮肤和粘膜的大疱性病变。病因是自身免疫的，与抗桥粒蛋白的IgG自身抗体的形成有关，它们是桥粒的结构蛋白，确保细胞之间接触的稳定性。天疱疮患者的心脏受累记录很少。我们报告了有关此主题的文献数据，以及一例天疱疮相关的自身免疫性心肌炎，并对免疫抑制治疗产生反应的插入盘受损。应通过心内膜活检适当筛查天疱疮患者的心肌病伴左心室功能障碍的发生，因为这可能是潜在可逆性自身免疫性疾病的心肌扩展。
