Clinical and demographic characteristics of mucous membrane pemphigoid in India: A retrospective analysis
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Published on: December 15, 2011
Dipankar De1, Vinod Hanumanthu1, Jitender Jinagal2
1Department of Dermatology, Venereology and Leprology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Abstract:
Background 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. Objective We aimed to study the clinical and demographic characteristics, disease course, and treatment aspects of MMP patients. Methods 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. Results 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. Limitations 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. Conclusion 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.
Insights
Mucous membrane pemphigoid (MMP) is a rare autoimmune blistering disease. This study highlights that MMP is not uncommon in India, with frequent visual complications requiring early diagnosis and treatment.
Area of Science:
- Dermatology
- Immunology
- Ophthalmology
Background:
- Mucous membrane pemphigoid (MMP) is a rare subepidermal autoimmune blistering disorder.
- Limited data exists on the clinical and demographic profiles of Indian MMP patients.
- Understanding these characteristics is crucial for effective disease management.
Purpose of the Study:
- To investigate the clinical and demographic features of MMP in Indian patients.
- To analyze the disease course and treatment modalities employed.
- To identify common complications and inform diagnostic and therapeutic strategies.
Main Methods:
- Retrospective analysis of case records from the Autoimmune Bullous Disease (AIBD) Clinic.
- Diagnosis confirmed by clinical and immune-histopathological features.
- Data collected on patient demographics, affected sites, disease progression, and treatment outcomes.
Main Results:
- 52 MMP patients were analyzed; mean age at onset was 50 years, with a female predominance (1.36:1).
- Oral (82.6%) and ocular (63.4%) mucosae were most frequently affected; 50% reported visual difficulty.
- Prednisolone was the primary treatment (44/52), often combined with steroid-sparing agents; Rituximab used for severe cases.
Conclusions:
- MMP appears more prevalent in India than previously reported.
- Visual complications are common, emphasizing the need for prompt diagnosis and management.
- Early detection and intervention are critical to prevent severe ocular sequelae.
