An immunogenetic study of bullous pemphigoid with mucosal involvement in two siblings

2015 ◽  
Vol 25 (2) ◽  
pp. 186-188 ◽  
Author(s):  
Akane Minagawa ◽  
Fuyuko Arakura ◽  
Hiroshi Koga ◽  
Yasutaka Tokuda ◽  
Hiroshi Koga ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Mucosal Involvement

Bullous pemphigoid as an injection site reaction of glatiramer acetate

2020 ◽  
Author(s):  
Seyed Mohammad Baghbanian ◽  
Maryam Ghasemi ◽  
Somayeh Sheidaei ◽  
Zohreh Hajheydari
Keyword(s):  
Injection Site ◽  
Glatiramer Acetate ◽  
Bullous Pemphigoid ◽  
Injection Site Reaction ◽  
Brain Magnetic Resonance Imaging ◽  
Skin Lesions ◽  
Mucosal Involvement ◽  
Disease Modifying ◽  
Magnetic Resonance Imaging Mri ◽  
Disease Modifying Treatment

Glatiramer acetate (GA) is one of the well-tolerated disease-modifying therapeutic options, which is commonly administered subcutaneously in patients with multiple sclerosis (MS). The current study aimed at defining a bullous pemphigoid (BP) skin reaction in a patient with MS receiving treatment with GA. A 29-year-old women with MS, receiving GA treatment within the past nine months, November 11, 2018, was admitted to our MS clinic due to itching skin eruptions at the site of injection. Her disease was started at February 12, 2017 with left optic neuritis; and because of six periventricular and eleven juxtacortical brain magnetic resonance imaging (MRI) lesions without any enhancement, lumbar puncture was done for her. Due to positive cerebrospinal fluid (CSF) oligoclonal band, MS was diagnosed for her. At that time, she refused to start disease modifying treatment. GA was started 9 months before admission for her.  After dermatologic consultation, the dermatologist defined the lesions as fluid-filled and blistering at the site of injection without mucosal involvement (Figure 1).   Figure 1. Large, fluid-filled blisters   Timeline: Skin lesions appeared exactly at the GA injection site.

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

scholarly journals Risk Factors for Mucosal Involvement in Bullous Pemphigoid and the Possible Mechanism: A Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Xinyi Chen ◽  
Wenlin Zhao ◽  
Hongzhong Jin ◽  
Li Li
Keyword(s):  
Risk Factors ◽  
Bullous Pemphigoid ◽  
Age At Onset ◽  
Mucous Membrane Pemphigoid ◽  
Dipeptidyl Peptidase 4 ◽  
Novel Treatments ◽  
Mucosal Involvement ◽  
Dipeptidyl Peptidase 4 Inhibitors ◽  
Potential Risk Factors ◽  
The Relationship

Bullous pemphigoid (BP) is the most common type of autoimmune bullous disease and is characterized by the presence of circulating anti-BP180 and/or anti-BP230 autoantibodies. Patients with BP often present with tense blisters and erythema, mainly on the trunk and limbs, but a few patients also have mucosal involvement. In this article, we discuss the fact that BP patients with mucosal involvement tend to have more serious conditions and their disease is more difficult to control. Potential risk factors for mucous involvement include earlier age at onset, drugs such as dipeptidyl peptidase-4 inhibitors, cancer, and blood/serum biomarkers, including lower eosinophil count, higher erythrocyte sedimentation rate, IgG autoantibodies against both the NH2- and COOH-termini of BP180, and the absence of anti-BP230 antibodies. IgA and C3 deposition at the dermo-epidermal junction may also be present. Understanding these risk factors may benefit earlier diagnosis of these patients and promote the development of novel treatments. What's more, it's helpful in deeper understanding of BP development and the relationship between BP and mucous membrane pemphigoid (MMP).

scholarly journals Evaluation of Recombinant Antigen-Based Assays for Diagnosis of Bullous Autoimmune Diseases

2004 ◽  
Vol 11 (4) ◽  
pp. 762-765 ◽  
Author(s):  
G. D'Agosto ◽  
A. Latini ◽  
M. Carducci ◽  
A. Mastroianni ◽  
A. Vento ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Mucous Membrane ◽  
Pemphigus Vulgaris ◽  
Clinical Aspects ◽  
Mucous Membrane Pemphigoid ◽  
Pemphigus Foliaceus ◽  
Serum Samples ◽  
Cutaneous Lesions ◽  
Autoimmune Bullous Diseases ◽  
Mucosal Involvement

ABSTRACT The diagnosis of autoimmune bullous diseases is based on clinical observation and on the presence of autoantibodies directed to molecules involved in the adhesion systems of the skin. Immunofluorescence assays are the currently accepted method for detection of autoantibodies; such assays depend greatly on the skill of operators and are difficult to standardize. Recombinant desmoglein-1 (Dsg1), Dsg3, and BP180 peptides, the main autoantigens in pemphigus or bullous pemphigoid, have been used to develop new quantitative enzyme immunoassays (EIA) for the detection of specific antibodies. The present study was undertaken to evaluate the sensitivity and specificity of these immunoassays and to determine the correlation between the results and the clinical aspects of diseases. Serum samples from patients with pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, or mucous membrane pemphigoid, from healthy individuals, and from patients with unrelated autoimmune conditions were tested. Anti-desmoglein reactivity was detected in all the patients with pemphigus and in none of the controls. Patients with the more benign form of cutaneous disease had anti-Dsg1 antibodies, while patients with deeper cutaneous lesions or with mucosal involvement had anti-Dsg3 reactivity also, or exclusively. The BP180-based assay was positive for 66.6% of patients with bullous pemphigoid and for none of the patients with mucous membrane pemphigoid, and no reactivity was detected in the control sera. In conclusion, the anti-Dsg1 and anti-Dsg3 assays are useful in the diagnosis of pemphigus and provide information on the clinical phenotype of the disease. However, the sensitivity of EIA for detection of autoantibodies in bullous pemphigoid should be improved by the use of additional antigens or epitopes.

scholarly journals A Case of Oropharyngeal Bullous Pemphigoid Presenting with Haemoptysis

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
C. M. Lee ◽  
H. K. Leadbetter ◽  
J. M. Fishman
Keyword(s):  
Elderly Patient ◽  
Bullous Pemphigoid ◽  
Soft Palate ◽  
Mucous Membrane Pemphigoid ◽  
Presenting Symptoms ◽  
Mucosal Involvement ◽  
Disease Entities ◽  
Symptoms And Signs ◽  
Nose And Throat ◽  
Aggressive Variant

Objective.Bullous pemphigoid is well known for its cutaneous features; however in rare cases it may present with mucosal involvement. We report a case of bullous pemphigoid presenting with haemoptysis, initially presenting to the Ear, Nose and Throat Department for investigation.Methods.An 87-year-old lady was admitted with haemoptysis. She also complained of a spreading, pruritic, bullous rash, which first began three weeks previously. Initial investigations, which included nasendoscopy, revealed a normal nasal mucosa and a normal postnasal space. A large deroofed blister was observed on the soft palate. The presenting symptoms and signs raised the suspicion of an immunobullous disease including bullous pemphigoid.Conclusion.Bullous pemphigoid (BP) is a subepidermal immunobullous disease that typically manifests in elderly patient populations. Although rare, BP can present in a mucocutaneous fashion akin to its more aggressive variant, mucous membrane pemphigoid (MMP). Differentiation of the two is based on clinical grounds, with the prevailing feature for the latter being the predominance of mucosal involvement, which may be extensive. The mainstay of treatment for bullous pemphigoid is steroid therapy, which may be administered both topically and systemically. A deeper understanding into the pathophysiology of the various immunobullous diseases may assist in our understanding of how the various disease entities manifest themselves.

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