Validation of theBIOCHIPtest for the diagnosis of bullous pemphigoid, pemphigus vulgaris and pemphigus foliaceus

AbstractBackgroundBoth enzyme-linked immunosorbent assays (ELISAs) and indirect immunofluorescence (IIF) are available for the diagnosis of autoimmune bullous diseases (AIBD). Many studies have reported on the performance of ELISAs and concluded that ELISAs could replace IIF. This study compares the diagnostic accuracy of ELISA and IIF for the detection of autoantibodies to desmoglein 1 (DSG1), desmoglein 3 (DSG3), bullous pemphigoid antigen 2 (BP180) and bullous pemphigoid antigen 1 (BP230) to support the diagnosis of pemphigus vulgaris (PV), pemphigus foliaceus (PF) and bullous pemphigoid (BP).MethodsA literature search was performed in the PubMed database. The meta-analysis was performed using summary values and a bivariate random effect model.ResultsThe five included studies on PV did not demonstrate significant differences between IIF and DSG3-ELISA (sensitivity 82.3% vs. 81.6%, p = 0.9284; specificity 95.6% vs. 93.9%, p = 0.5318; diagnostic odds ratio [DOR] 101.60 vs. 67.760, p = 0.6206). The three included studies on PF did not demonstrate significant differences between IIF and DSG1-ELISA (sensitivity 80.6% vs. 83.1%, p = 0.8501; specificity 97.5% vs. 93.9%, p = 0.3614; DOR 160.72 vs. 75.615, p = 0.5381). The eight included studies on BP showed that BP230-ELISA differed significantly from both IIF on monkey esophagus (MO) and BP180-ELISA with regard to DOR (11.384 vs. 68.349, p = 0.0008; 11.384 vs. 41.699, p = 0.0125, respectively)ConclusionsOur meta-analysis shows that ELISA performs as well as IIF for diagnosing PV, PF and BP.

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Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: A systematic review

Indian Journal of Dermatology Venereology and Leprology ◽

10.4103/0378-6323.82400 ◽

2011 ◽

Vol 77 (4) ◽

pp. 456 ◽

Cited By ~ 9

Author(s):

Sanjay Singh

Keyword(s):

Systematic Review ◽

Bullous Pemphigoid ◽

Pemphigus Vulgaris ◽

Pemphigus Foliaceus ◽

Evidence Based ◽

Evidence Based Treatments

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Bullous Skin Diseases: Classical Types of Autoimmune Diseases

Scientifica ◽

10.1155/2013/457982 ◽

2013 ◽

Vol 2013 ◽

pp. 1-5 ◽

Cited By ~ 3

Author(s):

Jan Damoiseaux

Keyword(s):

Autoimmune Diseases ◽

Bullous Pemphigoid ◽

Oral Mucosa ◽

Skin Diseases ◽

Pemphigus Vulgaris ◽

Pemphigus Foliaceus ◽

Blister Formation ◽

Koch’S Postulates ◽

Bullous Skin Diseases ◽

Koch's Postulates

The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin and/or oral mucosa in combination with circulating and deposited autoantibodies reactive with (hemi)desmosomes. Koch’s postulates, adapted for autoimmune diseases, were applied on these skin diseases. It appears that all adapted Koch’s postulates are fulfilled, and, therefore, these bullous skin diseases are to be considered classical autoimmune diseases within the wide and expanding spectrum of autoimmune diseases.

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Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment

Dermatology Practical & Conceptual ◽

10.5826/dpc.1003a50 ◽

2020 ◽

pp. e2020050 ◽

Cited By ~ 2

Author(s):

Vito Di Lernia ◽

Dahiana M. Casanova ◽

Mohamad Goldust ◽

Cinzia Ricci

Keyword(s):

Bullous Pemphigoid ◽

Pemphigus Vulgaris ◽

Screening Tools ◽

Enzyme Linked Immunosorbent Assay ◽

Pemphigus Foliaceus ◽

Line Treatment ◽

First Line ◽

Systemic Corticosteroids ◽

Autoimmune Bullous Diseases ◽

First Line Treatment

Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. The 2 major groups of diseases are “pemphigus diseases” and “autoimmune bullous diseases of the pemphigoid type.” Pemphigus diseases are a group of autoimmune blistering diseases of the skin and mucous membranes characterized by intraepithelial cleft and acantholysis. The main subtypes of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Diagnosis is based on clinical manifestations and confirmed with histological, immunofluorescence, and serological testing. Recently multivariant enzyme‐linked immunosorbent assay systems have been developed as practical screening tools for patients with suspected autoimmune bullous dermatoses. The current first-line treatment of pemphigus is based on systemic corticosteroids that are often combined with immunosuppressive adjuvants, such as azathioprine, mycophenolate mofetil, and the anti-CD20 monoclonal antibody rituximab, usually at initiation of treatment. Rituximab efficacy is higher when it is administered early in the course of the disease. Therefore, it should be used as first-line treatment to improve efficacy and reduce cumulative doses of corticosteroids and their side effects. Treatment of bullous pemphigoid is based on disease extension. Localized and mild forms can be treated with superpotent topical corticosteroids or with nonimmunosuppressive agents. In patients with generalized disease or whose disease is resistant to the treatments described above, systemic corticosteroids are preferred and effective. Adjuvant immunosuppressants are often combined with steroids for their steroid-sparing effect.

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Clinical, histopathological and immunofluorescent study of vesicobullous lesions of skin

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20191341 ◽

2019 ◽

Vol 7 (4) ◽

pp. 1288

Author(s):

Seeram Satish Kumar ◽

Bhagyalakshmi Atla ◽

Guru Prasad Patnala ◽

Kukkala Saraswati Sarat Srinivas ◽

Saraswathi Samantra ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Histopathological Examination ◽

Pemphigus Vulgaris ◽

Direct Immunofluorescence ◽

Pemphigus Foliaceus ◽

Female Ratio ◽

Medical College ◽

Intensive Investigation ◽

Male To Female

Background: Vesiculobullous diseases have been the focus of intensive investigation in recent years. However, these disorders are still associated with substantial morbidity, considerable mortality and impaired quality of life. Accurate diagnosis of vesiculobullous lesions of skin entails evaluation of clinical, histopathologic and immunofluorescence findings.Methods: Hospital based prospective study for a period of 24 months from August 2014 to July 2016 in the Department of Pathology at Andhra Medical College, Visakhapatnam, India. Total of 50 patients aged 3-70 years with vesiculobullous lesions of both sexes attending the Department of Dermatology were selected and analysed clinically, histopathological examination and direct immunofluorescence (DIF).Results: In the present study, majority of patients presented between 51-60 yrs of age (32%) with male to female ratio of 1.08:1 and mean age of 46.02 years. Pemphigus vulgaris constituted the most common vesiculobullous disorder (32%) followed by bullous pemphigoid and pemphigus foliaceous, 18% each. Bullae were located intra epidermally in 68% and sub epidermally in 32% of the patients. DIF was positive in 80% of the cases. Overall clinicopathological correlation was established in 74%. Overall histopathological and direct immunofluorescence correlation was established in 78%. Out of 50 cases, 35 cases (70%) correlated clinically and histo-pathologically with direct immunofluorescence.Conclusions: In the present study, on histopathological examination alone pemphigus foliaceus and pemphigus vulgaris could be differentiated. Direct immunofluorescence was useful in differentiating epidermolysis bullosa acquisita from bullous pemphigoid which have similar histopathological picture. This study proves that direct immunofluorescence is confirmatory as well as diagnostic for vesiculobullous disorders.

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Evaluation of Recombinant Antigen-Based Assays for Diagnosis of Bullous Autoimmune Diseases

Clinical and Diagnostic Laboratory Immunology ◽

10.1128/cdli.11.4.762-765.2004 ◽

2004 ◽

Vol 11 (4) ◽

pp. 762-765 ◽

Cited By ~ 4

Author(s):

G. D'Agosto ◽

A. Latini ◽

M. Carducci ◽

A. Mastroianni ◽

A. Vento ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Mucous Membrane ◽

Pemphigus Vulgaris ◽

Clinical Aspects ◽

Mucous Membrane Pemphigoid ◽

Pemphigus Foliaceus ◽

Serum Samples ◽

Cutaneous Lesions ◽

Autoimmune Bullous Diseases ◽

Mucosal Involvement

ABSTRACT The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Immunofluorescence assays are the currently accepted method for detection of autoantibodies; such assays depend greatly on the skill of operators and are difficult to standardize. Recombinant desmoglein-1 (Dsg1), Dsg3, and BP180 peptides, the main autoantigens in pemphigus or bullous pemphigoid, have been used to develop new quantitative enzyme immunoassays (EIA) for the detection of specific antibodies. The present study was undertaken to evaluate the sensitivity and specificity of these immunoassays and to determine the correlation between the results and the clinical aspects of diseases. Serum samples from patients with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, or mucous membrane pemphigoid, from healthy individuals, and from patients with unrelated autoimmune conditions were tested. Anti-desmoglein reactivity was detected in all the patients with pemphigus and in none of the controls. Patients with the more benign form of cutaneous disease had anti-Dsg1 antibodies, while patients with deeper cutaneous lesions or with mucosal involvement had anti-Dsg3 reactivity also, or exclusively. The BP180-based assay was positive for 66.6% of patients with bullous pemphigoid and for none of the patients with mucous membrane pemphigoid, and no reactivity was detected in the control sera. In conclusion, the anti-Dsg1 and anti-Dsg3 assays are useful in the diagnosis of pemphigus and provide information on the clinical phenotype of the disease. However, the sensitivity of EIA for detection of autoantibodies in bullous pemphigoid should be improved by the use of additional antigens or epitopes.

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Study of Various Vesiculobullous Lesions Using Tzanck Smear

Annals of Pathology and Laboratory Medicine ◽

10.21276/apalm.2791 ◽

2020 ◽

Vol 7 (6) ◽

pp. A301-305

Author(s):

Sanchit Singhal ◽

Hemalata M

Keyword(s):

Early Diagnosis ◽

Bullous Pemphigoid ◽

Viral Infections ◽

Pemphigus Vulgaris ◽

Giant Cells ◽

Pemphigus Foliaceus ◽

Dermoepidermal Junction ◽

Non Invasive ◽

Histopathological Correlation ◽

Invasive Method

Background: The vesiculobullous reaction pattern is characterized by the presence of vesicles or bullae within the epidermis or at the dermoepidermal junction. Despite some having characteristic presentations, it’s difficult to make a definite diagnosis clinically. Hence, cytological evaluation is required for reliable and early diagnosis. Objectives of the study are to determine the incidence of various vesiculobullous lesions and evaluate cytology as a tool for early diagnosis of vesiculobullous lesions. Methods: For Tzanck smears fresh vesicle or bulla was selected and incised with scalpel, reflecting the roof of bulla. Base of the blister scraped gently and material spread on a glass slide. These smears were stained with MGG (air dried), Pap and H&E stains (fixed).Skin punch biopsies from the bullae were subjected to routine fixation, processing, sectioning and staining. Results: A total of 62 Tzanck smears were done for vesiculobullous lesions of skin of which 29 had histopathological correlation. Herpes constituted the most common vesiculobullous disorder (42%) followed by bullous pemphigoid (27.4%) and pemphigus vulgaris (19.3%). Most patients were in the age group 61- 70 years. The M:F ratio of 1:1.38 showing female preponderance. Tzanck smears showed acantholytic cells in pemphigus group, eosinophils in bullous pemphigoid and multinucleate giant cells in viral blisters. Histopathology showed intraepidermal acantholysis in pemphigus vulgaris, subcorneal blister in pemphigus foliaceus and subepidermal in bullous pemphigoid. Conclusion: Cytohistopathological correlation showed an overall sensitivity of 79%. Tzanck smear showed 96% sensitivity for viral infections. Tzanck smear is a quick, non-invasive method for the early diagnosis of vesiculobullous disorders.

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