Clinical and demographic characteristics of mucous membrane pemphigoid in India: A retrospective analysis

Dipankar De, V. Hanumanthu, J. Jinagal, Sanjeev Handa, Rahul Mahajan, Debajyoti Chatterjee, Vinod Kumar, B. Saikia, U. Nahar Saikia, Bishan Dass Radotra, R. Minz
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Abstract

Mucous membrane pemphigoid (MMP) is a rare subepidermal autoimmune blistering disorder. The clinical and demographic parameters of this disease in Indian patients have not yet been elucidated in detail. We aimed to study the clinical and demographic characteristics, disease course, and treatment aspects of MMP patients. The data for this study were obtained by reviewing the case record forms of patients registered in the Autoimmune Bullous Disease (AIBD) Clinic of the Department of Dermatology, Venereology & Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, a tertiary care centre in India. The diagnosis of MMP was established on the basis of clinical and immune-histopathological features which are consistent with standard diagnostic criteria for the disease. A total of 52 patients with MMP registered in the AIBD clinic were included. The mean age at disease onset was 50 years and the average age at presentation was 56 years. Females outnumbered males in the study with a ratio of 1.36:1. The oral and ocular mucosae were the most commonly affected sites (82.6% and 63.4% respectively). Visual difficulty was reported by half the patients (26 of 52 patients). IgG, C3, and IgA deposits were detected on direct immunofluorescence (DIF) in 29, 21, and 11 patients, respectively. Serologic analysis was performed in only 7 of the patients and of these, just 1 exhibited a positive result on multivariant ELISA and epidermal pattern of binding on salt split skin indirect immunofluorescence. Most patients were treated with prednisolone (44 of 52). Steroid-sparing adjuvants were used in combination including cyclophosphamide, azathioprine, methotrexate, dapsone, and colchicine. Rituximab was administered in 7 patients with severe or refractory disease. This is a retrospective analysis of data available from a clinic registry. In patients with negative direct immunofluorescence on biopsy, the diagnosis was based on clinico-pathologic consensus. MMP is not as uncommon in India as the paucity of reports suggest. Visual complications are frequent in Indian MMP patients. A high index of suspicion is required for early diagnosis and appropriate treatment to prevent ocular complications.
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印度粘膜丘疹病的临床和人口特征:回顾性分析
粘膜丘疹性荨麻疹(MMP)是一种罕见的表皮下自身免疫性水疱病。我们旨在研究 MMP 患者的临床和人口统计学特征、病程和治疗方面的情况。本研究的数据是通过审查印度三级医疗中心昌迪加尔研究生医学教育与研究学院皮肤病、性病学和皮肤病学系自身免疫性大疱病(AIBD)门诊登记患者的病例记录表获得的。MMP 的诊断是根据临床和免疫组织病理学特征确定的,这些特征符合该疾病的标准诊断标准。患者发病时的平均年龄为 50 岁,发病时的平均年龄为 56 岁。口腔和眼部粘膜是最常受影响的部位(分别占 82.6% 和 63.4%)。直接免疫荧光法(DIF)分别在 29、21 和 11 例患者中检测到 IgG、C3 和 IgA 沉积。仅对 7 名患者进行了血清学分析,其中仅有 1 人在多变量 ELISA 和盐分皮肤间接免疫荧光的表皮结合模式中显示出阳性结果。大多数患者都接受了泼尼松龙治疗(52 例中有 44 例),并联合使用了节省类固醇的辅助药物,包括环磷酰胺、硫唑嘌呤、甲氨蝶呤、达帕松和秋水仙碱。7名病情严重或难治的患者使用了利妥昔单抗。对于活检直接免疫荧光呈阴性的患者,诊断依据的是临床病理学共识。印度的 MMP 患者经常出现视觉并发症。印度的 MMP 患者经常出现视力并发症,因此需要高度怀疑,以便及早诊断和适当治疗,防止出现眼部并发症。
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