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Turk Patoloji Dergisi 2016Sub-epidermal bullous disorders belong to immunobullous diseases which develop as a result of autoantibody action against epidermal basement membrane proteins....
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.
Topics: Adolescent; Adult; Aged; Autoimmune Diseases; Child; Female; Fluorescent Antibody Technique, Direct; Humans; Male; Middle Aged; Skin Diseases, Vesiculobullous; Tertiary Care Centers; Young Adult
PubMed: 27136107
DOI: 10.5146/tjpath.2015.01345 -
Acta Dermato-venereologica Jun 2016
Topics: Adult; Embryo Transfer; Enzyme-Linked Immunosorbent Assay; Female; Fluorescent Antibody Technique, Indirect; Glucocorticoids; Humans; Pemphigoid Gestationis; Prednisone; Pregnancy; Pregnancy Complications; Pregnancy Outcome
PubMed: 26608214
DOI: 10.2340/00015555-2303 -
Acta Dermato-venereologica Jan 2017
Topics: Adult; Diagnosis, Differential; Female; Humans; Immunologic Techniques; Pemphigoid Gestationis; Pregnancy; Sensitivity and Specificity
PubMed: 27174635
DOI: 10.2340/00015555-2460 -
SAGE Open Medical Case Reports 2019Polymorphic eruption of pregnancy, formerly known as pruritic and urticarial papules and plaques of pregnancy, is an uncommon cutaneous eruption that can affect women...
Polymorphic eruption of pregnancy, formerly known as pruritic and urticarial papules and plaques of pregnancy, is an uncommon cutaneous eruption that can affect women during their third trimester of pregnancy. As its name implies, it has a variety of morphologies and it is important to consider other diagnoses, such as pemphigoid gestationis, which polymorphic eruption of pregnancy can mimic. Sometimes, as in this case, polymorphic eruption of pregnancy can have a targetoid morphology reminiscent of erythema multiforme. A thorough workup and conservative management plan helps reassure the patient that the correct approach is being taken during the challenging period of a pregnancy nearing completion.
PubMed: 31662850
DOI: 10.1177/2050313X19882841 -
Cureus Mar 2024Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating...
Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating autoantibodies against antigens in the epidermis or the dermo-epidermal junction. Direct immunofluorescence (DIF) for immunoglobulin (Ig)G, IgC3, and IgA on fresh-frozen tissue is the gold standard diagnostic test for AIBDs. However, DIF in the absence of frozen tissue is challenging for the diagnosis of AIBDs. This study aimed to analyze the practical utility of DIF using paraffin-embedded skin biopsy rather than fresh frozen tissue for the diagnosis of AIBDs. Methodology This cross-sectional comparative study included 30 cases of AIBDs. DIF for IgG and IgA was performed on paraffin-embedded tissue (PE-DIF) after proteinase digestion on histopathologically confirmed 15 pemphigus vulgaris (PV), three pemphigus foliaceous (PF), four bullous pemphigoid (BP), three dermatitis herpetiformis (DH), three subcorneal pustular dermatosis (SCPD), and one case each of linear IgA disease and pemphigoid gestationis (PG). PE-DIF staining pattern was compared with the DIF on fresh frozen tissue (FF-DIF). Results All cases of PV and PF showed an intercellular IgG chicken wire staining pattern similar to FF-DIF. However, background staining was more intense in PV cases while less intense in PF cases. Three BP cases showed linear IgG staining in PE-DIF. DH, SCPD, linear IgA disease, and PG cases did not show IgG positivity. Out of three DH cases, two cases showed granular IgA positivity while linear IgA positivity along the basement membrane was seen in a single case of linear IgA disease. Negative IgG staining was observed in SCPD. Immunofluorescence in PE-DIF was rapidly deteriorating than in FF-DIF. Conclusions DIF done on paraffin-embedded tissue can be used as a supplement and salvage technique with histopathology for the diagnosis of AIBDs, particularly when a cryostat facility for frozen tissue is not available and the patient is unable to undergo a second biopsy procedure.
PubMed: 38665766
DOI: 10.7759/cureus.56916 -
JAAD Case Reports Apr 2024
PubMed: 38463656
DOI: 10.1016/j.jdcr.2024.01.026 -
Clinical, Cosmetic and Investigational... 2018Autoimmune subepidermal bullous dermatoses have similar clinical features to those of a spectrum of immune reactants at the dermoepidermal junction (DEJ). It is...
BACKGROUND
Autoimmune subepidermal bullous dermatoses have similar clinical features to those of a spectrum of immune reactants at the dermoepidermal junction (DEJ). It is difficult to obtain a precise diagnosis without an immunofluorescence assay because of their similar clinical presentations. The aim of this study was to describe the cellular cutaneous infiltration among autoimmune subepidermal bullous dermatoses.
MATERIALS AND METHODS
This retrospective analysis was conducted at a hospital in Riyadh, Saudi Arabia using biopsy-based data collected from 65 patients.
RESULTS
Spongiotic changes, neutrophils, and lymphocyte infiltrations in the epidermis differed among the subepidermal bullous diseases. The DEJ showed a difference in the extent of neutrophil infiltration. The dermis showed differences in perivascular lymphocytic infiltration, neutrophilic infiltration, eosinophilic infiltration, and dermal edema.
CONCLUSION
The dermal and DEJ showed most of the histopathologic changes in subepidermal autoimmune bullous dermatoses.
PubMed: 29731653
DOI: 10.2147/CCID.S158388 -
Actas Dermo-sifiliograficas Jan 2021
Topics: Female; Humans; Immunoglobulins, Intravenous; Pemphigoid Gestationis; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Pregnancy
PubMed: 32986975
DOI: 10.1016/j.ad.2019.04.016 -
Acta Dermato-venereologica Sep 2015
Elevated serum levels of BP180 antibodies in the first trimester of pregnancy precede gestational pemphigoid and remain elevated for a long time after remission of the disease.
Topics: Autoantibodies; Autoantigens; Biomarkers; Case-Control Studies; Enzyme-Linked Immunosorbent Assay; Female; Humans; Non-Fibrillar Collagens; Pemphigoid Gestationis; Predictive Value of Tests; Pregnancy; Pregnancy Trimester, First; Recurrence; Remission Induction; Time Factors; Treatment Outcome; Up-Regulation; Collagen Type XVII
PubMed: 25758329
DOI: 10.2340/00015555-2088 -
The Pan African Medical Journal 2014
Topics: Adult; Ectodermal Dysplasia; Female; Glucocorticoids; Humans; Infant, Newborn; Pemphigoid Gestationis; Pregnancy
PubMed: 25667709
DOI: 10.11604/pamj.2014.19.47.4914