An Erythrodermic Variant of Pemphigus Foliaceus with Puzzling Histologic and Immunopathologic Features

2007 ◽  
Vol 11 (5) ◽  
pp. 179-184 ◽  
Author(s):  
Jennifer D. Peterson ◽  
Sophie M. Worobec ◽  
Lawrence S. Chan

Background: Pemphigus foliaceus is an autoimmune blistering disorder that affects the skin owing to autoantibodies against desmoglein 1. Methods: We employed clinical, histologic, immunopathologic, and serum laboratory studies to investigate a case of an erythrodermic variant of pemphigus foliaceus in an elderly man following treatment with bisoprolol-hydrochlorothiazide. Results: Early histopathology revealed psoriasiform dermatitis, but later biopsies showed subcorneal and granular layer separation with neutrophilic infiltrate. Direct immunofluorescence showed intercellular deposits of immunoglobulin G throughout the epidermis, granular staining of C3 along the basement membrane zone, and fibrin and C3 deposition around the blood vessels. Indirect immunofluorescence on monkey esophagus showed a titer of greater than 1:1,280. Indirect immunofluorescence on rat bladder, antinuclear antibody, lupus panel, and kidney function panel were all negative. Conclusion: There are no reports in the literature of pemphigus foliaceus being induced by bisoprolol, but reports exist of propanolol resulting in drug-induced pemphigus foliaceus.

1996 ◽  
Vol 33 (3) ◽  
pp. 332-336 ◽  
Author(s):  
T. Iwasaki ◽  
M. Shimizu ◽  
H. Obata ◽  
M. Ogata ◽  
M. Nagata ◽  
...  

The effect of substrate on indirect immunofluorescence (IIF) tests for the detection of circulating autoantibodies was studied by examining sera from 14 canine pemphigus foliaceus patients, six sera with non-pemphigus dermatoses and ten normal dog sera against five different substrates from three species. These substrates included bovine esophagus, bovine nose, bovine tongue, monkey esophagus, and canine nose skin. Nine out of 14 (64.3%) sera from patients with canine pemphigus foliaceus showed intercellular space staining by indirect immunofluorescence using bovine esophagus as substrate. However, sera from nonpemphigus dermatoses and normal dog did not react with bovine esophagus. In other substrates, only bovine tongue showed 1/8 (12.5%) positive reaction at the intercellular space by sera from canine pemphigus foliaceus. Dog nose skin showed the intercellular space staining against ten of ten (100%) normal dog serum. Monkey esophagus showed the fluorescent deposit at the intercellular space in four of nine (44.4%) of pemphigus foliacues dog sera, however, four of ten (40%) of normal dog sera revealed nonspecific intercellular staining. These results indicate that the sensitivity and the specifity of IIF test in canine pemphigus foliaceus depend on the substrate. The best substrate for detecting circulating autoantibody in canine pemphigus foliaceus patients among five different substrates was bovine esophagus because of its sensitivy and high specifity. The diagnosis of canine pemphigus foliaceus should be made on the basis of a combination of clinical signs, histopathology, direct immunofluorescence, and the detection of circulating autoantibody.


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.


Author(s):  
Silvia Regina C. Sartori Barravieira ◽  
Neuza Lima Dillon ◽  
Maria Tereza Rezkallah-Iwasso ◽  
Maria Terezinha Serrão Peraçoli ◽  
Paulo Roberto Curi ◽  
...  

A study was conducted on 16 patients with pemphigus foliaceus, ten of them with the localized form (group G1) and six with the disseminated form (group G2). These patients were submitted to full blood counts, quantitation of mononuclear cell subpopulations by monoclonal antibodies, study of blastic lymphocyte transformation, and quantitation of circulating antibodies by the indirect immunofluorescence test, in order to correlate their clinical signs and symptoms and laboratory data with their immunological profile, and to determine the relationship between circulating autoantibody titers and lesion intensity and course of lesions under treatment. Leucocytosis was observed especially in group G2. All patients showed decreased relative CD3+ and CD4+ values and a tendency to decreased relative values of the CD8+ subpopulation. Blastic lymphocyte transformation indices in the presence of phytohemagglutinin were higher in patients (group G1+G2) than in controls. The indirect immunofluorescence test was positive in 100% of G2 patients and in 80% of G1 patients. The median value for the titers was higher in group G2 than in group G1. Analysis of the results as a whole permits us to conclude that cell immunity was preserved and that there was a relationship between antibody titers detected by the direct immunofluorescence test and extent of skin lesions.


2001 ◽  
Vol 144 (2) ◽  
pp. 421-422 ◽  
Author(s):  
K. Ogata ◽  
H. Nakajima ◽  
M. Ikeda ◽  
Y. Yamamoto ◽  
M. Amagai ◽  
...  

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):  
Gavin P Spickett

Introduction Techniques: overview Particle agglutination assays Immunoprecipitation assays Indirect immunofluorescence Direct immunofluorescence Radio-immunoassay (RIA) Enzyme-linked (EIA) and fluorescent immunoassays (FIA) Immunoblotting Acetylcholine-receptor antibodies (AChRAb) Actin antibodies Adrenal cortex autoantibodies Amphiphysin antibodies Anti-nuclear antibodies (ANA) and ANCA Aquaporin antibodies Auerbach’s plexus antibodies β‎2-GPI antibodies C1q antibodies...


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