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 ◽  
...  

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.

2013 ◽  
Vol 17 (5) ◽  
pp. 321-331 ◽  
Author(s):  
Daisuke Tsuruta ◽  
Amrinder J. Kanwar ◽  
Keshavamurthy Vinay ◽  
Shunpei Fukuda ◽  
Hiroshi Koga ◽  
...  

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.


2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Marta Sar-Pomian ◽  
Lidia Rudnicka ◽  
Malgorzata Olszewska

Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution in hair follicles, as well as information about clinical manifestations, histopathology, immunopathology, and trichoscopy of scalp lesions in pemphigus and their response to treatment.


Author(s):  
Anuradha Jindal ◽  
Chythra Rao ◽  
Satish B. Pai ◽  
Raghavendra Rao

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.


2021 ◽  
Vol 97 (2) ◽  
pp. 9-15
Author(s):  
Alexey V. Samtsov ◽  
Evgeny V. Sokolovskiy ◽  
Natalia P. Teplyuk ◽  
Irena E. Belousova ◽  
Muza M. Kokhan ◽  
...  

Analysis of various classifications of pemphigus shows that there are no fundamental differences between them. The main distinctions consist in use of diverse terms in naming of some forms of pemphigus and in inclusion or exclusion of certain subtypes from the classifications. Authors propose to use the following classification in the dermatological clinical practice, for educational and scientific purposes and for clinical guidelines: 1) pemphigus vulgaris (1.1. Pemphigu s vegetans); 2) pemphigus foliaceus (2.1. Pemphigus endemic (Fogo selvagem), 2.2. Pemphigus erythematosus (Senear Usher)); 3) herpetiform pemphigus; 4) paraneoplastic pemphigus; 5) IgA pemphigus (5.1. Subcorneal pustular dermatosis, 5.2. Intraepidermal neutrophilic dermatosis).


2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Hossein Mortazavi ◽  
Alireza Khatami ◽  
Zahra Seyedin ◽  
Iman Vasheghani Farahani ◽  
Maryam Daneshpazhooh

Background. Serum desmoglein enzyme-linked immunosorbent assay (ELISA) is used for the diagnosis and monitoring of pemphigus diseases.Objectives. To compare the diagnostic accuracy of salivary antidesmoglein (Dsg) 1 and 3 ELISA in the diagnosis of pemphigus vulgaris (PV) patients with that of serum desmogleins ELISA.Methods. Eighty-six untreated PV patients and 180 age- and sex-matched PV-free controls were recruited in this case-control study. PV was diagnosed based on clinical, histopathological, and direct immunofluorescence findings. After processing, serum and salivary anti-Dsg 1 and 3 were measured by the ELISA method using Euroimmun kit (Lübeck, Germany).Results. Using the cut-off point of 20 relative units (RU)/mL, the serum anti-Dsg 1 and 3 ELISA were positive in 62 (72.1%) and 83 (96.5%) patients, respectively, and the salivary anti-Dsg 1 and 3 ELISA were positive in 31 (36.1%) and 63 (73.3%) patients, respectively. The specificity of salivary anti-Dsg 1 and anti-Dsg 3 were both 98.9%. Optimal cut-off values of 7.7 and 13.4 RU/mL were determined for the salivary anti-Dsg 1 and anti-Dsg 3 ELISA, respectively.Conclusion. Salivary anti-Dsg 1 and 3 ELISA with high specificities (98.9%) could be suggested as safe and noninvasive methods for the diagnosis of PV when obtaining a blood sample is difficult.


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