Clinical and Immunologic Characterization in 26 Indian Pemphigus Patients

2013 ◽  
Vol 17 (5) ◽  
pp. 321-331 ◽  
Author(s):  
Daisuke Tsuruta ◽  
Amrinder J. Kanwar ◽  
Keshavamurthy Vinay ◽  
Shunpei Fukuda ◽  
Hiroshi Koga ◽  
...  
Keyword(s):  
Clinical Features ◽  
Immunosorbent Assay ◽  
Indirect Immunofluorescence ◽  
Pemphigus Vulgaris ◽  
Enzyme Linked Immunosorbent Assay ◽  
Pemphigus Foliaceus ◽  
Lower Sensitivity ◽  
Before And After ◽  
Desmoglein 3

Background: Pemphigus shows geographically variable characteristics. Objective: To study the clinical and immunologic characteristics of Indian pemphigus patients before and after treatment. Methods: Twenty-six Indian pemphigus patients were analyzed with regard to age, gender, clinical features, treatments and response, the results of histopathology, direct and indirect immunofluorescence (IF), enzyme-linked immunosorbent assay (ELISA), and immunoblot analyses. Results: There were 22 pemphigus vulgaris (PV) and 4 pemphigus foliaceus (PF) patients. Direct and indirect IF was positive in 95.8% and 56% of patients, respectively. Indices of ELISA were lower in our study. Immunoblot assays detected the 130 kDa desmoglein-3 in 10 PV patients and the 160 kDa desmoglein-1 in 1 PV patient; 190 kDa periplakin was unexpectedly detected in 8 patients. Conclusion: Indian pemphigus patients showed several unique characteristics, including younger population, predominance of PV, low ELISA indices, lower sensitivity of indirect IF and immunoblotting, and the presence of the 190 kDa periplakin in nearly one-third of patients.

scholarly journals Utility of oral mucosa as a substrate for the serodiagnosis of pemphigus: A descriptive analysis

2021 ◽  
Vol 0 ◽  
pp. 1-6
Author(s):  
Anuradha Jindal ◽  
Chythra Rao ◽  
Satish B. Pai ◽  
Raghavendra Rao
Keyword(s):  
Human Skin ◽  
Oral Mucosa ◽  
Immunosorbent Assay ◽  
Indirect Immunofluorescence ◽  
Pemphigus Vulgaris ◽  
Enzyme Linked Immunosorbent Assay ◽  
Pemphigus Foliaceus ◽  
Normal Human Skin ◽  
Normal Human ◽  
Monkey Esophagus

Background: The indirect immunofluorescence test is useful in the serodiagnosis of pemphigus. As indirect immunofluorescence titers correlate with disease activity in pemphigus, it is often used as a monitoring tool. The sensitivity of indirect immunofluorescence depends on the substrate used, and the preferred substrates are monkey esophagus for pemphigus vulgaris and normal human skin for pemphigus foliaceus. Aims: We evaluated oral mucosa as a substrate for indirect immunofluorescence in pemphigus. Methods: Fifty patients with pemphigus (40 with pemphigus vulgaris and ten with pemphigus foliaceus) and 50 controls were enrolled for study. Demographic and clinical details were recorded and indirect immunofluorescence using two substrates (oral mucosa and normal human skin) was carried out in serial dilution. Desmoglein (Dsg) 1 and 3 enzyme-linked immunosorbent assay was also evaluated simultaneously. Results: Indirect immunofluorescence was positive in 40 patients (80%) with oral mucosa substrate and 34 patients (68%) with normal human skin substrate. Circulating antibodies were detected with oral mucosa in 33 (82.5%) of the 40 pemphigus vulgaris patients and in 26 (65%) patients using normal human skin. Antibodies were detected in eight of the ten pemphigus foliaceus patients (80%) with normal human skin and in seven (70%) patients with oral mucosa. Dsg enzyme-linked immunosorbent assay was positive in 45 (90%) patients, and 37 of these were also indirect immunofluorescence positive with oral mucosa. In the five Dsg enzyme-linked immunosorbent assay-negative patients, indirect immunofluorescence with oral mucosa was positive in three. Limitations: A comparison of oral mucosa with monkey esophagus could not be performed. Conclusion: Oral mucosa is a suitable and sensitive substrate for indirect immunofluorescence in pemphigus. Further studies comparing the sensitivity of indirect immunofluorescence using oral mucosa with monkey esophagus are recommended.

scholarly journals Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

2015 ◽  
Vol 1 (2) ◽  
Author(s):  
Dario Didona ◽  
Biagio Didona ◽  
Antonio G Richetta ◽  
Carmen Cantisani ◽  
Elisa Moliterni ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Clinical Features ◽  
Immunosorbent Assay ◽  
Indirect Immunofluorescence ◽  
Laboratory Tests ◽  
Diagnostic Methods ◽  
Enzyme Linked Immunosorbent Assay ◽  
Paraneoplastic Pemphigus ◽  
Pathological Evaluation

<p style="margin: 0cm 0cm 8pt; text-align: justify; line-height: 150%;">Paraneoplastic pemphigus is a rare autoimmune disease of the skin associated with neoplasm. Nowadays, the pathogenesis of paraneoplastic pemphigus is not fully understood. Due to its rarity, various criteria have been proposed for the diagnosis. For this reason, several diagnostic methods have been considered useful for the diagnosis of paraneoplastic pemphigus including indirect immunofluorescence, direct immune of fluorescence, immunoprecipitation, immunoblotting, and enzyme-linked immunosorbent assay (ELISA). However, the polymorphic clinical features and the various results of laboratory tests and pathological evaluation present a challenge for the clinicians.</p>

scholarly journals An Updated Review of Pemphigus Diseases

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1080
Author(s):  
Ali M. Malik ◽  
Sarah Tupchong ◽  
Simo Huang ◽  
Abhirup Are ◽  
Sylvia Hsu ◽  
...  
Keyword(s):  
Clinical Features ◽  
Clinical Assessment ◽  
Treatment Options ◽  
Delayed Diagnosis ◽  
Pemphigus Vulgaris ◽  
Enzyme Linked Immunosorbent Assay ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Iga Pemphigus ◽  
Anti Cd20

Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed.

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