Pemphigus and Bullous Pemphigoid

2020 ◽  
Author(s):  
Aakaash Varma ◽  
Annette Czernik ◽  
Jacob Levitt
Keyword(s):  
Botulinum Toxin ◽  
Bullous Pemphigoid ◽  
Clinical Features ◽  
Indirect Immunofluorescence ◽  
Direct Immunofluorescence ◽  
Topical Steroids ◽  
Bladder Epithelium ◽  
Paraneoplastic Pemphigus ◽  
Rat Bladder ◽  
Collagen Xvii

Pemphigus disorders are characterized by acantholysis, whereas pemphigoid disorders are characterized by a dermal-epidermal split. Diagnosis of pemphigus or pemphigoid relies on a combination of positive anti-desmoglein or anti-collagen XVII serology, confirmatory direct immunofluorescence, and clinical features. Treatment for immunobullous disease revolves around various immunosuppressants, most often some combination of rituximab, prednisone, and IVIg. Paraneoplastic pemphigus is characterized by hemorrhagic crusting of the lips with positive indirect immunofluorescence on rat bladder epithelium, which should prompt a search for malignancy. Hailey-Hailey disease is a genetically mediated pemphigus that typically occurs in skin folds and responds to a number of agents including botulinum toxin, topical steroids, and other anecdotal therapies. This review contains 17 figures, 2 tables, and 109 references. Keywords: blister, pemphigus, bullous, rituximab, bullae, prednisone

Pemphigus and Bullous Pemphigoid

2020 ◽  
Author(s):  
Aakaash Varma ◽  
Annette Czernik ◽  
Jacob Levitt
Keyword(s):  
Botulinum Toxin ◽  
Bullous Pemphigoid ◽  
Clinical Features ◽  
Indirect Immunofluorescence ◽  
Direct Immunofluorescence ◽  
Topical Steroids ◽  
Bladder Epithelium ◽  
Paraneoplastic Pemphigus ◽  
Rat Bladder ◽  
Collagen Xvii

Pemphigus disorders are characterized by acantholysis, whereas pemphigoid disorders are characterized by a dermal-epidermal split. Diagnosis of pemphigus or pemphigoid relies on a combination of positive anti-desmoglein or anti-collagen XVII serology, confirmatory direct immunofluorescence, and clinical features. Treatment for immunobullous disease revolves around various immunosuppressants, most often some combination of rituximab, prednisone, and IVIg. Paraneoplastic pemphigus is characterized by hemorrhagic crusting of the lips with positive indirect immunofluorescence on rat bladder epithelium, which should prompt a search for malignancy. Hailey-Hailey disease is a genetically mediated pemphigus that typically occurs in skin folds and responds to a number of agents including botulinum toxin, topical steroids, and other anecdotal therapies. This review contains 17 figures, 2 tables, and 109 references. Keywords: blister, pemphigus, bullous, rituximab, bullae, prednisone

scholarly journals Paraneoplastic pemphigus: A trait d’union between dermatology and oncology

2015 ◽  
Vol 1 (2) ◽  
Author(s):  
Dario Didona ◽  
Biagio Didona ◽  
Antonio G Richetta ◽  
Carmen Cantisani ◽  
Elisa Moliterni ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Clinical Features ◽  
Immunosorbent Assay ◽  
Indirect Immunofluorescence ◽  
Laboratory Tests ◽  
Diagnostic Methods ◽  
Enzyme Linked Immunosorbent Assay ◽  
Paraneoplastic Pemphigus ◽  
Pathological Evaluation

<p style="margin: 0cm 0cm 8pt; text-align: justify; line-height: 150%;">Paraneoplastic pemphigus is a rare autoimmune disease of the skin associated with neoplasm. Nowadays, the pathogenesis of paraneoplastic pemphigus is not fully understood. Due to its rarity, various criteria have been proposed for the diagnosis. For this reason, several diagnostic methods have been considered useful for the diagnosis of paraneoplastic pemphigus including indirect immunofluorescence, direct immune of fluorescence, immunoprecipitation, immunoblotting, and enzyme-linked immunosorbent assay (ELISA). However, the polymorphic clinical features and the various results of laboratory tests and pathological evaluation present a challenge for the clinicians.</p>

Indirect immunofluorescence on rat bladder transitional epithelium: A test with high specificity for paraneoplastic pemphigus

1993 ◽  
Vol 28 (5) ◽  
pp. 696-699 ◽  
Author(s):  
Aries Y. Liu ◽  
Rafael Valenzuela ◽  
Thomas N. Helm ◽  
Charles Camisa ◽  
Alton L. Melton ◽  
...  
Keyword(s):  
Indirect Immunofluorescence ◽  
High Specificity ◽  
Transitional Epithelium ◽  
Paraneoplastic Pemphigus ◽  
Rat Bladder

scholarly journals Localized Bullous Pemphigoid on the Site of Knee Arthroplasty: A Case Report

2016 ◽  
Vol 8 (1) ◽  
pp. 39-44 ◽  
Author(s):  
Lucija Kosi ◽  
Jelena Perić ◽  
Milica Pantović ◽  
Gorana Bijelić ◽  
Jelica Vukićević Sretenović ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Knee Arthroplasty ◽  
Skin Lesions ◽  
Direct Immunofluorescence ◽  
Topical Steroids ◽  
Cutaneous Lesions ◽  
Predisposing Factor ◽  
Dermatological Examination ◽  
The Right ◽  
Split Skin

Abstract Localized bullous pemphigoid is a rare variant of bullous pemphigoid, and its exact etiopathogenesis is yet to be elucidated. We present a case of a 74-year-old Caucasian male with a 3-month history of skin lesions that appeared 9 months after he underwent a knee arthroplasty. Dermatological examination showed several pruritic tense bullae on the right knee, localized around the surgical scar, as well as erosions covered with crusts. The diagnosis of localized bullous pemphigoid was confirmed by direct immunofluorescence test (conventional and split-skin). The patient was treated with potent topical steroids, which led to complete resolution of cutaneous lesions. We suppose that the occurrence of localized bullous pemphigoid in our patient may be explained by the concept of “immunocompromised district” in which one disease (surgery) caused an immunological alteration which is a predisposing factor for the development of secondary disease such as localized bullous pemphigoid.

scholarly journals Localised bullous pemphigoid overlying knee arthroplasty: a diagnostic challenge

2019 ◽  
Vol 12 (4) ◽  
pp. e227440
Author(s):  
Adam Truss ◽  
Stylianos Papalexandris ◽  
Susan Gardner ◽  
Robert Harvey
Keyword(s):  
Bullous Pemphigoid ◽  
Knee Arthroplasty ◽  
Clinical Findings ◽  
Direct Immunofluorescence ◽  
Caucasian Woman ◽  
Topical Steroids ◽  
Diagnostic Challenge ◽  
Soft Tissue Swelling ◽  
Total Knee ◽  
Allergic Contact

An elderly Caucasian woman developed bullous pemphigoid (BP) overlying the site of total knee arthroplasty for osteoarthritis 2 days after surgery. The clinical findings were consistent with blistering due to soft tissue swelling, bullous impetigo or allergic contact dermatitis. The blistering spread over weeks to months down the ipsilateral leg and then to the other leg and hips. A biopsy for H&E and direct immunofluorescence established the diagnosis of BP. A concomitantly occurring wound infection was identified and treated. The blistering responded well to superpotent topical steroids and local care.

scholarly journals Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate

Clinics ◽  
2011 ◽  
Vol 66 (12) ◽  
pp. 2019-2023 ◽  
Author(s):  
Damaris G. Ortolan ◽  
Danielle P. G. Souza ◽  
Valéria Aoki ◽  
Claudia G. Santi ◽  
Tatiana V. B. Gabbi ◽  
...  
Keyword(s):  
Indirect Immunofluorescence ◽  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Bladder Epithelium ◽  
Rat Bladder
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