Ding Shu, Deng Qiancheng, Xiang Yaping, Chen Jing, Huang Jinhua, Lu Jianyun
Department of Dermatology, Third Xiangya Hospital, Central South University, Changsha, China.
An Bras Dermatol. 2017;92(5 Suppl 1):34-36. doi: 10.1590/abd1806-4841.20176124.
Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.
大疱性类天疱疮是一种以两种半桥粒蛋白（抗BP180和230）为特征的水疱性自身免疫性疾病。相比之下，天疱疮的特征是两种自身抗体（抗桥粒芯糖蛋白1和3）。患者同时存在大疱性类天疱疮和天疱疮的自身抗体情况罕见。一名25岁男性患者入住我院，自述全身广泛红斑上出现多个丘疹、水疱及糜烂3个月。实验室检查显示血清IgE、抗BP180抗体以及抗桥粒芯糖蛋白1和3水平升高。组织病理学和免疫病理学特征符合大疱性类天疱疮。然而，全身糖皮质激素治疗未见改善，而脉冲糖皮质激素联合甲泼尼龙、免疫抑制剂、免疫调节剂及血浆置换治疗使病情恢复，出现大量粟丘疹。
