Li Si-Zhe, Xie Ying-Han, Wang Si-Hang, Fang Rou-Yu, Jin Hong-Zhong, Zuo Ya-Gang
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Front Med (Lausanne). 2022 Dec 16;9:1023458. doi: 10.3389/fmed.2022.1023458. eCollection 2022.
Lichen planus pemphigoides (LPP) is a rare autoimmune bullous disease, characterized by the coexistence of lichen planus and subepidermal bullae. However, the minority of LPP patients present with papules rather than vesicles or blisters, which is defined as non-bullous LPP. The diagnosis of LPP relies on manifestations, histopathology, serological assay, and direct immunofluorescence of linear disposition of IgG and/or C3 at the basement membrane zone. Up to now, no standard therapeutic strategies have been proposed for the treatment of LPP. Herein, we describe an uncommon non-bullous LPP patient with widespread papules and erythema, probably induced by vaccination. During hospitalization, he had a poor response to the conventional treatment of topical and systemic corticosteroids, and his condition was finally alleviated by the addition of dupilumab. For LPP patients with a traditional medication failure, or who were not suitable for a higher dose of corticosteroids, a combination with dupilumab could be an alternative option.
扁平苔藓类天疱疮（LPP）是一种罕见的自身免疫性大疱性疾病，其特征为扁平苔藓和表皮下大疱并存。然而，少数LPP患者表现为丘疹而非水疱或大疱，这被定义为非大疱性LPP。LPP的诊断依赖于临床表现、组织病理学、血清学检测以及免疫球蛋白G（IgG）和/或补体C3在基底膜带呈线性分布的直接免疫荧光检查。到目前为止，尚未提出治疗LPP的标准治疗策略。在此，我们描述了一名罕见的非大疱性LPP患者，其有广泛的丘疹和红斑，可能由接种疫苗诱发。住院期间，他对局部和全身使用皮质类固醇的传统治疗反应不佳，最终通过加用度普利尤单抗病情得到缓解。对于传统药物治疗失败或不适合使用更高剂量皮质类固醇的LPP患者，联合使用度普利尤单抗可能是一种替代选择。
