Concordance of Clinical, Histological and Direct Immunofluorescence Findings in Patients with Autoimmune Bullous Dermatosis
DOI:
https://doi.org/10.52206/jsmc.2025.15.1.961Abstract
Background: Autoimmune blistering disease (AIBD) is characterized by autoantibodies directed against various proteins in the skin and mucous membrane that ultimately results in blister formation. For the diagnosis of these diseases, combination of clinical presentation, histopathological and direct immunofluorescence (DIF) features are required.
Objective: To evaluate the concordance among clinical characteristics, histopathological and direct immunofluorescence findings of various immunobullous diseases.
Materials and Methods: This cross sectional descriptive study was conducted at Dermatology Department, Dow University of Health Sciences / Dr. Ruth Pfau Civil Hospital, Karachi, Pakistan from January 2019 to December 2023. Total 120 patients were included, and they were evaluated clinically and subsequently skin biopsy was performed for confirmation with histopathology and DIF.
Results: In our study, the predominant immunobullous disorder was Pemphigus vulgaris accounting for 53.3% (64/120) of cases followed by Pemphigus foliaceous at 19.2%. In the sub-epidermal group, Bullous pemphigoid, Dermatitis Herpetiformis, Linear IgA disease, Bullous SLE, EBA, Pemphigoid gestationis, and LP pemphigoides, each accounted for 10.8%, 6.7%, 4.2%, 2.5%, 1.7%, 0.8% and 0.8% of the total cases, respectively. Histopathological analysis revealed intraepidermal split in 72.5% of the cases, whereas subepidermal split was observed in 27.5% of the cases. Direct immunofluorescence was performed in 120 cases, yielding positive results in 115 cases while 5 cases were negative, demonstrating a sensitivity of 95.8%. A concordance of 95.8% was seen between clinical and histopathological findings, 91.6% between clinical and DIF findings, and 95.8% between histopathological and DIF findings.
Conclusion: Several Immunobullous diseases share similar histopathologic features, which can lead to overlap in diagnoses. Direct immunofluorescence (DIF) plays a critical role in distinguishing between these conditions.
Keywords: Autoimmune blistering diseases, Bullous pemphigoid, Concordance, Correlation, Dermatitis Herpetiformis, Direct immunofluorescence, Histopathology, Linear IgA disease, Pemphigus vulgaris, Pemphigus foliaceous.
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