Utility of direct immunofluorescence studies in subclassification of autoimmune sub-epidermal bullous diseases: a 2-year study in a tertiary care hospital

Abstract

Objective: sub-epidermal bullous disorders belong to immunobullous diseases which develop as a result of autoantibody action against epidermal basement membrane proteins. Clinically, they are tense bullae and do not rupture easily. They are classified into various forms based on histopathology and direct immunofluorescence patterns. This study was undertaken to assess the incidence of various sub-epidermal bullous disorders and the utility of direct immunofluorescence in accurately classifying them, and to study the intensity and pattern of immunofluorescence in various sub-epidermal bullous disorders Material and Method: a 2-year study of 38 cases of sub-epidermal bullous disorders sent for direct immunofluorescence studies formed the study group. The specimens were processed as per standard protocols. The clinical details were obtained from case files and requisition sent for histopathological and direct immunofluorescence studies.Results: Thirty-eight patients were diagnosed to have sub-epidermal bullous disorders over the period of 2 years. Twenty five cases of Bullous Pemphigoid, 5 cases of Dermatitis Herpetiformis, 3 cases of linear iga Bullous disorder, 2 cases of Bullous systemic lupus Erythematoses and 1 case each of Epidermolysis Bullosa acquisita, Cicatricial Pemphigoid and Pemphigus gestationis was diagnosed. Positive direct immunofluorescence was seen in 91.3% of the cases. Conclusion:Histopathology alone cannot classify sub-epidermal bullous disorders and direct immunofluorescence studies are mandatory in all of them. Bullous Pemphigoid needs to be distinguished from Epidermolysis Bullosa acquisita which requires salt split direct immunofluorescence studies. Dermatitis Herpetiformis, Bullous systemic lupus Erythematosus and linear iga Bullous disorder show more or less similar histological picture with neutrophilic microabscess. Direct immunofluorescence studies help in the majority of cases but further testing such as immunoblotting, immunoelectron microscopy or indirect immunofluorescence becomes essential in cases with overlapping features.

A retrospective study of dermatitis herpetiformis from an immunobullous disease clinic in north India

“…6,7 There have been few case reports from India, but no study has characterized clinical and histopathological characteristics of Indian DH patients. [11][12][13] De et al reported 14 patients of DH from our center (data of these patients has been included in this study), and suggested DH may not be as uncommon in India as previously thought. 14 Two major reasons for apparent low prevalence of DH in Asians can be more consumption of rice and absence of HLA-DQ2 and DQ8.…”

“…Dermatitis herpetiformis is a relatively rare disease with high prevalence in Scandinavian countries and in the USA . There have been few case reports from India, but no study has characterized clinical and histopathological characteristics of Indian DH patients . De et al .…”

Histologic characterization of cellular infiltration in autoimmune subepidermal bullous diseases in a tertiary hospital in Saudi Arabia

“…In this study, 72% of the patients were in the pemphigoid group, which was consistent with the findings of international studies. 10 Although it is difficult to give a precise estimate of the disease incidence of other subepidermal autoimmune bullous dermatoses because of their rarity, an annual incidence rate ranging from 0.26 to 0.67 cases per million people per year has been reported for LAD, BLE, and EBA. 11 – 14 The incidence of DH ranges from 11.5 to 39.2 cases per 100,000 people.…”

Direct Immunofluorescence in Cutaneous and Systemic Lupus Erythematosus: A Literature Review

“…60,61 The correlation between BSLE and systemic involvements including lupus nephritis, neuropsychiatric SLE and hematologic abnormality have been documented. 62 DIF studies of BSLE are usually performed in the perilesional area with clinical non-lesional skin to demonstrate staining of IgG, IgM, C3 and IgA at the DEJ with linear or granular patterns 35,59,[62][63][64][65][66][67][68][69][70][71][72][73][74]. The positive yield from the DIF test was high, at approximately 85.7%-100%.…”

“…The positive yield from the DIF test was high, at approximately 85.7%-100%. 35,59,[62][63][64][65][66][67][68][69][70][71][72][73][74] IgG was the most reported immunoreactant, while IgA was more common in BSLE than other forms of lupus. 65 Furthermore, IgA deposits were associated with disease activity.…”