scholarly journals Circulating Eosinophil and Neutrophil Counts Correlate with Disease Severity in Bullous Pemphigoid

2018 ◽  
Vol 30 (5) ◽  
pp. 544
Author(s):  
Seh Hyun Park ◽  
Si-Hyung Lee ◽  
Jong Hoon Kim ◽  
Soo-Chan Kim
Keyword(s):  
Disease Severity ◽  
Bullous Pemphigoid

scholarly journals Assessing the Correlation Between Autoimmune Bullous Disease Quality of Life Questionnaires (ABQOL, TABQOL, DLQI) and Disease Severity Indices in Pemphigus and Bullous Pemphigoid

2020 ◽  
Author(s):  
Shan Cao ◽  
Lulu Sun ◽  
Zhongxiang Shi ◽  
Baoqi Yang ◽  
Furen Zhang
Keyword(s):  
Quality Of Life ◽  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Strong Correlations ◽  
Disease Area ◽  
Area Index ◽  
Severity Of Disease ◽  
Bullous Disease ◽  
Autoimmune Bullous Disease

Abstract Background: Pemphigus and bullous pemphigoid (BP) are autoimmune blistering diseases (AIBDs) that affect the skin and mucous membranes, and adversely impact quality of life (QOL). Few studies have assessed the correlation between the severity of disease and QOL in patients with pemphigus and BP. Objectives: To identify the correlation between the severity of AIBDs and QOL using the Autoimmune Bullous Disease Quality of Life (ABQOL), Treatment Autoimmune Bullous Disease Quality of Life (TABQOL), and Dermatology Life Quality Index (DLQI) questionnaires in Chinese patients with pemphigus and BP at baseline, and after 1, 3 and 6 months of treatment. Methods: Pemphigus and BP patients were invited to complete the ABQOL, TABQOL, and DLQI questionnaires. We measured the pemphigus disease area index (PDAI), autoimmune bullous skin disorder intensity score (ABSIS), and antibodies of desmoglein1,desmoglein3 (DSG1/DSG3) for pemphigus; and the bullous pemphigoid disease area index (BPDAI), ABSIS, and antibodies of BP180/ BP230 for BP as disease severity indices. The correlations between the severity of disease and QOL were analyzed by Spearman’s correlation coefficient (r). Results: Eighty-five patients were included: 55 with pemphigus and 30 with BP. The pemphigus subtypes included pemphigus vulgaris (PV, n = 32), pemphigus foliaceus (PF, n = 22), and paraneoplastic pemphigus (PNP, n = 1). We found significantly strong correlations between QOL (ABQOL, TABQOL, DLQI) and severity of disease (PDAI/BPDAI, ABSIS) with (r = 0.87, 0.77, 0.83; r = 0.86, 0.73, 0.80) for pemphigus and BP, respectively. Mild or strong correlations were also observed between QOL and antibody titers in pemphigus with DSG1/DSG3(r=0.32/0.36) and BP with BP180/BP230( r = 0.73/0.17) respectively. Conclusion: The QOL of patients with pemphigus and BP decreased with increased severity of the AIBDs. As the disease severity descended, so the QOL improved. The QOL indices should be used in clinical trials and to manage patients’ treatment, especially during the active disease stage, despite the mild correlation observed after 1 month of treatment.The PDAI to be better at assessing disease severity than the ABSIS in patients with pemphigus, and ABSIS are better than BPDAI in BP patients for correlation with the QOL indices.

scholarly journals Correlation of dermal and blood eosinophilia with bullous pemphigoid disease severity

2020 ◽  
Vol 6 (3) ◽  
pp. 171-175
Author(s):  
Farshad Farnaghi ◽  
Amir-hooshang Ehsani ◽  
Kambiz Kamyab-Hesary ◽  
Sepideh Abbasian ◽  
Hasan Seirafi ◽  
...  
Keyword(s):  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Blood Eosinophilia

scholarly journals HSAN-VI

2020 ◽  
Vol 6 (1) ◽  
pp. e389 ◽  
Author(s):  
Anisha Lynch-Godrei ◽  
Rashmi Kothary
Keyword(s):  
Mouse Model ◽  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Autonomic Neuropathy ◽  
Genetic Disorder ◽  
Specific Effect ◽  
Sensory Neuropathy ◽  
Compensatory Mechanisms ◽  
Initial Characterization

Hereditary sensory and autonomic neuropathy (HSAN-VI) is a recessive genetic disorder that arises because of mutations in the human dystonin gene (DST, previously known as bullous pemphigoid antigen 1). Although initial characterization of HSAN-VI reported it as a sensory neuropathy that was lethal in infancy, we now know of a number of heterozygous mutations in DST that result in milder forms of the disease. Akin to what we observe in the mouse model dystonia musculorum (Dstdt), we believe that the heterogeneity of HSAN-VI can be attributed to a number of dystonin isoforms that the mutation affects. Lack of neuronal isoform dystonin-a2 is likely the universal determinant of HSAN-VI because all reported human cases are null for this isoform, as are all Dstdt mouse alleles. Compensatory mechanisms by intact dystonin-a isoforms also likely play a role in regulating disease severity, although we have yet to determine what specific effect dystonin-a1 and dystonin-a3 have on the pathogenesis of HSAN-VI.

Tissue eosinophil levels as a marker of disease severity in bullous pemphigoid

2021 ◽  
Author(s):  
Muge Gore Karaali ◽  
Ayse Esra Koku Aksu ◽  
Merve Cin ◽  
Cem Leblebici ◽  
Asude Kara Polat ◽  
...  
Keyword(s):  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Tissue Eosinophil

scholarly journals Mucosal Involvement in Bullous Pemphigoid Is Mostly Associated with Disease Severity and to Absence of Anti-BP230 Autoantibody

2018 ◽  
Vol 9 ◽  
Author(s):  
Ariane Clapé ◽  
Céline Muller ◽  
Grégory Gatouillat ◽  
Sébastien Le Jan ◽  
Coralie Barbe ◽  
...  
Keyword(s):  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Mucosal Involvement

Basement membrane zone IgE deposition is associated with bullous pemphigoid disease severity and treatment results

2019 ◽  
Vol 182 (5) ◽  
pp. 1221-1227 ◽  
Author(s):  
A. Kamata ◽  
Y. Kurihara ◽  
T. Funakoshi ◽  
H. Takahashi ◽  
K. Kuroda ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Disease Severity ◽  
Bullous Pemphigoid ◽  
Basement Membrane Zone ◽  
Treatment Results
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