Atypical clinical variants, predisposing factors and new therapeutic guidelines in bullous pemphigoid

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease in elderly people. The disease typically manifests on the skin and the mucosa with tense bullae or erosions with surrounding erythema, but in up to 20% of afected patients, bullae may be completely absent. The localized or generalized skin lesions usually present with itch. The author presents the rare, atypical forms of the disease, the most common triggering and predisposing factors, and the new therapeutic recommendations according to the European guidelines.

Download Full-text

Bullous Pemphigoid: Trigger and Predisposing Factors

Biomolecules ◽

10.3390/biom10101432 ◽

2020 ◽

Vol 10 (10) ◽

pp. 1432

Author(s):

Francesco Moro ◽

Luca Fania ◽

Jo Linda Maria Sinagra ◽

Adele Salemme ◽

Giovanni Di Zenzo

Keyword(s):

Bullous Pemphigoid ◽

Surgical Procedures ◽

Human Leukocyte ◽

Predisposing Factors ◽

Trigger Factor ◽

Trigger Factors ◽

Leukocyte Antigen ◽

Disease Identification ◽

Blistering Disease ◽

Electrical Burns

Bullous pemphigoid (BP) is the most frequent autoimmune subepidermal blistering disease provoked by autoantibodies directed against two hemidesmosomal proteins: BP180 and BP230. Its pathogenesis depends on the interaction between predisposing factors, such as human leukocyte antigen (HLA) genes, comorbidities, aging, and trigger factors. Several trigger factors, such as drugs, thermal or electrical burns, surgical procedures, trauma, ultraviolet irradiation, radiotherapy, chemical preparations, transplants, and infections may induce or exacerbate BP disease. Identification of predisposing and trigger factors can increase the understanding of BP pathogenesis. Furthermore, an accurate anamnesis focused on the recognition of a possible trigger factor can improve prognosis by promptly removing it.

Download Full-text

Retrospective Analysis of Checkpoint Inhibitor Therapy-Associated Cases of Bullous Pemphigoid From Six German Dermatology Centers

Frontiers in Immunology ◽

10.3389/fimmu.2020.588582 ◽

2021 ◽

Vol 11 ◽

Author(s):

Christian D. Sadik ◽

Ewan A. Langan ◽

Ralf Gutzmer ◽

Maria Isabel Fleischer ◽

Carmen Loquai ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Clinical Presentation ◽

Checkpoint Inhibitors ◽

Corticosteroid Treatment ◽

Time Interval ◽

European Guidelines ◽

Blistering Disease ◽

Checkpoint Inhibitor Therapy ◽

Ci Therapy ◽

Treatment Responses

Immune-related adverse events (irAEs) are a class-effect of checkpoint inhibitors (CIs). The development of a Bullous pemphigoid (BP)-like blistering disease, driven by autoantibodies against the hemidesmosomal protein BP180, is a potentially serious irAE whose incidence seems to be increasing. We therefore set out to characterize the clinical and (immuno)histopathological features and treatment responses of cases of BP which developed during or after CI therapy collated in six German tertiary referral centers between 2014 and 2018. We identified twelve cases of BP which emerged during and/or after CI therapy. The time interval between the initiation of CI therapy and the diagnosis of BP was 3–74 weeks (median: 23 weeks). Age at the time of diagnosis of BP varied between 62 and 80 years (median: 76 years). The clinical presentation of the patients was diverse but the severity was relatively mild when compared to that seen in most cases of spontaneous BP. Only four patients met all of the immunopathological criteria recommended in the European guidelines for the diagnosis of BP. Topical corticosteroid treatment was sufficient to achieve disease control in most patients. CI therapy could be continued in 8 out of 12 patients. In summary, our study indicates that cases of BP during or after CI therapy bear several peculiarities distinguishing them from spontaneous BP. Given the diversity of the clinical presentation of CI-induced BP the application of existing diagnostic algorithms developed for spontaneous BP can be utilized to uncover the frequency and features of CI-induced BP and to develop and optimize management algorithms.

Download Full-text

Do Guidelines for the Diagnosis and Monitoring of Diabetes Mellitus Fulfill the Criteria of Evidence-Based Guideline Development?

Clinical Chemistry ◽

10.1373/clinchem.2008.109082 ◽

2008 ◽

Vol 54 (11) ◽

pp. 1872-1882 ◽

Cited By ~ 17

Author(s):

Eva Nagy ◽

Joseph Watine ◽

Peter S Bunting ◽

Rita Onody ◽

Wytze P Oosterhuis ◽

...

Keyword(s):

Diabetes Mellitus ◽

Methodological Quality ◽

Guideline Development ◽

Evidence Based ◽

Test Results ◽

Time Period ◽

Therapeutic Guidelines ◽

Therapeutic Recommendations ◽

Evidence Based Guideline

Abstract Background: Although the methodological quality of therapeutic guidelines (GLs) has been criticized, little is known regarding the quality of GLs that make diagnostic recommendations. Therefore, we assessed the methodological quality of GLs providing diagnostic recommendations for managing diabetes mellitus (DM) and explored several reasons for differences in quality across these GLs. Methods: After systematic searches of published and electronic resources dated between 1999 and 2007, 26 DM GLs, published in English, were selected and scored for methodological quality using the AGREE Instrument. Subgroup analyses were performed based on the source, scope, length, origin, and date and type of publication of GLs. Using a checklist, we collected laboratory-specific items within GLs thought to be important for interpretation of test results. Results: The 26 diagnostic GLs had significant shortcomings in methodological quality according to the AGREE criteria. GLs from agencies that had clear procedures for GL development, were longer than 50 pages, or were published in electronic databases were of higher quality. Diagnostic GLs contained more preanalytical or analytical information than combined (i.e., diagnostic and therapeutic) recommendations, but the overall quality was not significantly different. The quality of GLs did not show much improvement over the time period investigated. Conclusions: The methodological shortcomings of diagnostic GLs in DM raise questions regarding the validity of recommendations in these documents that may affect their implementation in practice. Our results suggest the need for standardization of GL terminology and for higher-quality, systematically developed recommendations based on explicit guideline development and reporting standards in laboratory medicine.

Download Full-text

Characterization of the skin microbiota in bullous pemphigoid patients and controls reveals novel microbial indicators of disease

10.1101/2021.04.30.21256380 ◽

2021 ◽

Author(s):

Meriem Belheouane ◽

Britt M Hermes ◽

Nina Van Beek ◽

Sandrine Benoit ◽

Philippe Bernard ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Disease Risk ◽

Disease Status ◽

The Elderly ◽

Future Research ◽

Sampling Effort ◽

Skin Microbiota ◽

Important Indicator ◽

Blistering Disease ◽

Microbe Interactions

Bullous pemphigoid (BP) is an autoimmune skin blistering disease afflicting mostly the elderly and is associated with significantly increased mortality. Here, we conducted the most extensive sampling effort of skin microbiota in BP to date to analyze whether intra-individual, body-site-specific, and/or geographical variation contributes to the emergence of BP. We find marked differences in the skin microbiota of BP patients compared to that of control subjects, and moreover that disease status rather than skin biogeography governs the skin microbiota composition in BP. Our data reveal a discernible transitional stage between normal and diseased skin in BP characterized by a loss of protective microbiota and an increase in sequences matching Staphylococcus aureus, a known inflammation-promoting species. Notably, S. aureus is ubiquitously associated with disease status, suggesting that this taxon is an important indicator of BP. Importantly, differences in a few key indicator taxa are able to reliably discriminate between BP patients and controls, characterized by their opposing abundance patterns. This may serve as valuable information for assessing disease risk and treatment outcomes. Future research will focus on the functional analysis of host-microbe and microbe-microbe interactions and the relevance of the host genome for microbiota abundances to identify novel BP treatment approaches.

Download Full-text

Gender, clinical and topographic features of the localized scleroderma lesions in anogenital area

Russian Journal of Skin and Venereal Diseases ◽

10.17816/dv48930 ◽

2020 ◽

Vol 23 (3) ◽

pp. 165-173

Author(s):

Elena S. Snarskaya ◽

Yulia A. Semenchak

Keyword(s):

Clinical Symptoms ◽

Skin Lesions ◽

Localized Scleroderma ◽

Steady Increase ◽

Family Doctors ◽

Topographic Features ◽

Anogenital Area ◽

Clinical Variants ◽

Limited Scleroderma ◽

And Gender

Background: Currently, there is an increase in the number of skin lesions of anogenital localization, which is a silent epidemic, both due to the steady increase in the incidence of this pathology, and the interdisciplinary aspect of this problem. Materials and methods: In the article, the authors first analyzed and presented the data of clinical and morphological analysis of 104 patients with various clinical variants of limited scleroderma, on the basis of which the main phenotypic and gender-specific clinical and topographical features of anogenital zone lesions in this group of patients are presented. Results: Scleroatrophic lichen is one of the clinical variants of limited scleroderma, which is characterized by damage to the mucous membranes of the external genitals in both women and men. Lesions of such localization are late and often mistakenly diagnosed by specialists of related disciplines (obstetricians, gynecologists, urologists, family doctors, allergists, cosmetologists), which leads to high risks of developing genitourenal syndrome. Conclusions: The development of scleroatrophic lesions in the anogenital zone is accompanied by pronounced clinical symptoms, including: itching, pain of varying intensity, dysuria, dyspareunia and significant sexual dysfunction.

Download Full-text

Leprosy in Refugees and Migrants in Italy and a Literature Review of Cases Reported in Europe between 2009 and 2018

Microorganisms ◽

10.3390/microorganisms8081113 ◽

2020 ◽

Vol 8 (8) ◽

pp. 1113

Author(s):

Anna Beltrame ◽

Gianfranco Barabino ◽

Yiran Wei ◽

Andrea Clapasson ◽

Pierantonio Orza ◽

...

Keyword(s):

Infectious Disease ◽

Wide Spectrum ◽

Case Reports ◽

Case Series ◽

Skin Lesions ◽

Clinical Suspicion ◽

High Risk Population ◽

European Guidelines ◽

Eye Involvement ◽

Multibacillary Leprosy

Leprosy is a chronic neglected infectious disease that affects over 200,000 people each year and causes disabilities in more than four million people in Asia, Africa, and Latin America. The disease can appear with a wide spectrum of clinical forms, and therefore the clinical suspicion is often difficult. Refugees and migrants from endemic countries affected by leprosy can remain undiagnosed in Europe due to the unpreparedness of clinicians. We retrospectively describe the characteristics of 55 refugees/migrants with a diagnosis of leprosy established in Italy from 2009 to 2018. Continents of origin were Africa (42%), Asia (40%), and South and Central America (18%). The symptoms reported were skin lesions (91%), neuropathy (71%), edema (7%), eye involvement (6%), fever (6%), arthritis (4%), and lymphadenopathy (4%). Seven patients (13%) had irreversible complications. Overall, 35% were relapses and 66% multibacillary leprosy. Furthermore, we conducted a review of 17 case reports or case series and five nationwide reports, published in the same decade, describing 280 migrant patients with leprosy in Europe. In Europe, leprosy is a rare chronic infectious disease, but it has not completely disappeared. Diagnosis and treatment of leprosy in refugees and migrants from endemic countries are a challenge. European guidelines for this neglected disease in this high-risk population would be beneficial.

Download Full-text