A Clinico-Epidemiological Study of Various Autoimmune Vesiculobullous Disorders in a Tertiary Care Center

TL;DR

This study examines the clinical and epidemiological features of autoimmune blistering skin disorders in a hospital in India, highlighting their prevalence, demographics, and treatment outcomes.

Contribution

The study provides a detailed analysis of AIBD cases in a specific Indian tertiary care center over seven years, focusing on demographic and treatment patterns.

Findings

Females (56%) were slightly more affected than males (44%), with the highest prevalence in the 51-60 age group.

Pemphigus vulgaris and bullous pemphigoid were the most common types, and most patients were from rural, low-income backgrounds.

Corticosteroids were the primary treatment, with some patients benefiting from DCP and rituximab.

Abstract

Background

Pemphigus and pemphigoid disorders, as well as dermatitis herpetiformis, are all part of the diverse group of conditions known as autoimmune vesiculobullous/blistering disorders (AIBDs). These disorders are caused by autoantibodies that target intercellular or cell-matrix adhesion proteins and are clinically characterized by blisters or erosions of the skin and/or mucous membranes. The incidence peaks at 30-60 years with an equal incidence in men and women. There are 0.1 to 0.5 cases of pemphigus for every 100,000 people.

Materials and methods

This single-center retrospective observational study was conducted at our tertiary care center (Great Eastern Medical School and Hospital) in Srikakulam, Andhra Pradesh, India, between March 2018 and March 2025, and examined 50 histopathologically and direct immunofluorescence confirmed cases of autoimmune vesiculobullous disorders over a seven-year period.

Results

The percentage of females, i.e., 28 (56%) out of 50 individuals, is marginally higher than that of males (22 (44%)). The most prevalent age group is 51-60 years old (26%), followed by 41-50 years old (20%). The majority of cases are from rural areas (72%) and are occupied by people from low socioeconomic backgrounds (72%). The majority of farmers are local residents (76%) with few migrants (24%). Of the 50 cases, the most prevalent intraepidermal variants were pemphigus vulgaris (18 cases) and pemphigus foliaceus (nine cases), with a single case of pemphigus vegetans, whereas the most prevalent sub-epidermal variants were bullous pemphigoid (14 cases), with a small number of epidermolysis bullosa acquisita cases (eight cases). In 44% of instances, mucosal involvement is observed. Comorbidities, such as diabetes, hypertension, heart disease, and malignancies, were found in a small percentage of cases. Corticosteroids were the first line of treatment, and immunosuppressants were used for maintenance. Few patients had positive outcomes using dexamethasone cyclophosphamide pulse therapy (DCP), and rituximab given in two cases showed disease control after the second dose. Better disease control was demonstrated by patients who adhered to the long-term treatment.

Conclusion

In recent years, autoimmune vesiculobullous illnesses have become more prevalent and are significantly correlated with other systemic conditions. A difficult problem that requires careful handling is atypical clinical manifestations, which can be linked to inappropriate and delayed treatment as well as noncompliance. Long-term steroid medication is less necessary with early diagnosis, which further minimizes consequences by evaluating the disease activity and severity and starting a targeted treatment. For the general improvement of quality of life, public education, development of medical services in rural regions, and the elimination of social stigma are, therefore, crucial.

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