1,862 results match your criteria Cicatricial Pemphigoid


Case Report: Mucous Membrane Pemphigoid With IgG and IgA Anti-Laminin γ1 Antibodies and IgA Anti-Laminin α5 Antibodies.

Front Immunol 2022 1;13:903174. Epub 2022 Jun 1.

Dermatology Hospital of Jiangxi Province, Jiangxi Provincial Clinical Research Center for Skin Diseases, Candidate Branch of National Clinical Research Center for Skin Diseases, Dermatology Institute of Jiangxi Province, The Affiliated Dermatology Hospital of Nanchang University, Nanchang, China.

Mucous membrane pemphigoid (MMP) and anti-laminin (LM) γ1 pemphigoid, two subtypes of subepidermal autoimmune bullous diseases characterized by autoantibodies against epidermal basement membrane zone proteins, mainly show mucosal and skin lesions, respectively. The known autoantigens of MMP includes BP180, BP230, LM332, integrin α6β4 and type VII collagen, and anti-LMγ1 pemphigoid targets LMγ1. In this study, we present an unique MMP case with oral mucosal lesions, which showed positive IgA signals on basement membrane zone in indirect immunofluorescence using normal human skin and on dermal side in indirect immunofluorescence using salt-split skin, positive IgA autoantibodies against LMγ1 by immunoblotting of epidermal extracts, positive IgA autoantibodies against LMα5 by immunoblotting of LM521 recombinant protein (rLM521) and positive IgG autoantibodies against LMγ1 by immunoblotting of rLM111 and rLM521 at first visit (Day 0). Read More

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Utility of Direct Immunofluorescence using buccal mucosal biopsies in those with suspected isolated Ocular Mucous Membrane Pemphigoid.

Ophthalmology 2022 Jun 7. Epub 2022 Jun 7.

Departments of Dermatology and Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, 75390. Electronic address:

Purpose: To determine the rate of positivity of immunofluorescence studies in buccal biopsies in patients with cicatrizing conjunctivitis undergoing workup for Ocular Mucous Membrane Pemphigoid/ocular cicatricial pemphigoid (OCP).

Design: Retrospective cohort review PARTICIPANTS: Forty-one patients with cicatrizing conjunctivitis undergoing workup for OCP METHODS: A retrospective chart review of direct immunofluorescence studies in buccal mucosal biopsies was performed.

Main Outcome Measures: Primary outcome measure was the rate of positivity of direct and indirect immunofluorescence studies on buccal mucosal biopsies. Read More

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Platelet-rich fibrin a new approach in management of persistent oral ulcers in blistering skin diseases.

Wound Repair Regen 2022 May 19. Epub 2022 May 19.

Faculty of Dentistry, Cairo University, Cairo, Egypt.

The present study designed to evaluate the healing power of platelet-rich fibrin (PRF) in terms of pain control and mucosal repair. A randomised, controlled, pilot clinical trial was conducted on 16 patients randomly distributed with 1:1 allocation ratio into two groups. The treatment group received PRF minced and mixed with orabase and the control group received clobetasol propionate 0. Read More

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Single-Staged Surgical Correction of Eyelid Sequelae Along With Lid Margin Mucous Membrane Grafting in Steven-Johnson Syndrome and Other Cicatricial Ocular Surface Diseases.

Cornea 2022 Apr 21. Epub 2022 Apr 21.

Cornea and Anterior Segment Services, Dr Shroff's Charity Eye Hospital, New Delhi, India.

Purpose: The purpose of this study was to study the outcome of single-staged entropion surgery along with lid margin mucous membrane grafting for cicatrizing ocular surface disease.

Methods: Retrospective review of medical records of patients who underwent single-staged surgical correction of cicatricial entropion along with lid margin mucous membrane grafting for lid margin keratinization.

Results: Twenty-six eyes of 19 patients were studied. Read More

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Perioperative Management and Long-Term Outcomes in Ocular Cicatricial Pemphigoid Patients Undergoing Cataract Surgery.

Oxid Med Cell Longev 2022 30;2022:2496649. Epub 2022 Apr 30.

Department of Ophthalmology, Peking University Third Hospital, Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Peking University Third Hospital, Beijing 100191, China.

Objective: To observe the outcomes of cataract surgery in ocular cicatricial pemphigoid (OCP) patients and explore routine perioperative medical treatments.

Design: Retrospective case series.

Methods: Fourteen eyes of 8 patients were included in the study. Read More

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Rapid Disease Control in First-Line Therapy-Resistant Mucous Membrane Pemphigoid and Bullous Pemphigoid with Omalizumab as Add-On Therapy: A Case Series Of 13 Patients.

Front Immunol 2022 20;13:874108. Epub 2022 Apr 20.

Department of Dermatology and Referral Center for Autoimmune Bullous Diseases (MALIBUL), Avicenne Hospital, Hôpitaux Universitaires de Paris Seine-Saint-Denis, AP-HP, Université Sorbonne Paris Nord, Bobigny, France.

The role of IgE autoantibodies has been demonstrated in the pathogenesis of bullous pemphigoid for many years. Recently, omalizumab (OMZ), a humanized monoclonal anti-IgE antibody that depletes total serum IgE, has been used off-label in a few case series of bullous pemphigoids demonstrating a rapid efficacy and allowing significant improvements or complete remission as add-on therapy in first-line treatment-resistant patients. Herein, we report the largest retrospective study to evaluate OMZ effectiveness in patients with subepidermal autoimmune blistering diseases. Read More

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Multifaceted mucous membrane pemphigoid.

Authors:
Warren R Heymann

J Am Acad Dermatol 2022 07 2;87(1):46-47. Epub 2022 May 2.

Division of Dermatology, Cooper Medical School of Rowan University, Camden, New Jersey. Electronic address:

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Szemészeti érintettséggel járó paraneoplasiás pemphigoid.

Orv Hetil 2022 May 1;163(18):720-725. Epub 2022 May 1.

1 Semmelweis Egyetem, Általános Orvostudományi Kar, Szemészeti Klinika Budapest, Mária u. 39., 1085 Magyarország.

Összefoglaló. A nyálkahártya-pemphigoid különleges formája a paraneoplasiás pemphigoid, amely primer malignus betegséghez társul; lefolyása szokatlanul gyors, és a klasszikus immunszuppresszív terápiákkal szemben rezisztens lehet. Közleményünkben három eseten keresztül mutatjuk be a paraneoplasiás pemphigoid megjelenését, diagnosztikáját és a terápiás kihívásokat. Read More

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Evaluation of the SUN Classification Criteria for Uveitides in an Academic Uveitis Practice.

Am J Ophthalmol 2022 Apr 22;241:57-63. Epub 2022 Apr 22.

From the Department of Ophthalmology, University of Colorado School of Medicine (L.I.M., A.K.R., J.L.P., P.P., E.K., K.C., A.G.P.), 1675 Aurora Ct, Aurora, CO, USA. Electronic address:

Purpose: To evaluate the new Standardization of Uveitis Nomenclature (SUN) classification criteria for uveitides by applying them to patients in an academic uveitis practice.

Design: Evaluation of classification criteria.

Methods: The charts of all patients attending the uveitis service at the University of Colorado Hospital between January 1, 2013, and December 31, 2020, were reviewed. Read More

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Clinical and Serological Characterization of Orf-Induced Immunobullous Disease.

JAMA Dermatol 2022 Jun;158(6):670-674

Department of Dermatology, University of Lübeck, Lübeck, Germany.

Importance: Ecthyma contagiosum, or orf, is a viral zoonotic infection caused by Poxviridae. Although human orf infection is considered to follow a self-limited course, various immunological reactions may be triggered, including immunobullous diseases. In the majority of the latter cases, the antigenic target remained enigmatic. Read More

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Management of bilateral conjunctival squamous carcinoma following ocular cicatricial pemphigoid: A case report and review of literature.

Eur J Ophthalmol 2022 Mar 29:11206721221090698. Epub 2022 Mar 29.

Operational Unit of Ophthalmology, 18560St Anna University Hospital of Ferrara, Ferrara, Italy.

The purpose of this study is to report a case of bilateral highly locally invasive conjunctival squamous neoplasia in the clinical setting of ocular cicatricial pemphigoid (OCP), and to review the available literature about this rare association. A man presented with chronic bilateral conjunctivitis and forniceal foreshortening. He had been previously diagnosed with OCP, and received proper therapy with systemic Dapsone and local steroids. Read More

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Drug induced cicatrizing conjunctivitis: A case series with review of etiopathogenesis, diagnosis and management.

Ocul Surf 2022 04 2;24:83-92. Epub 2022 Mar 2.

Centre for Ocular Regeneration, L V Prasad Eye Institute, Hyderabad, Telangana, India; The Cornea Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India. Electronic address:

Drug induced cicatrizing conjunctivitis (DICC) is defined as a disease in which conjunctival cicatrization develops as a response to the chronic use of inciting topical and, rarely, systemic medications. DICC accounts for up to one third of cases of pseudopemphigoid, a large group of cicatrizing conjunctival diseases sharing similar clinical features to those of mucous membrane pemphigoid (MMP) but generally without the morbidity of progressive scarring or the need for systemic immunosuppression. The preservatives in topical anti-glaucoma medications (AGM) are the most frequently implicated inciting causes of DICC although topical antivirals, vasoconstrictors and mydriatics and some systemic drugs have been implicated. Read More

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Reply Re: "Direct Injection of 5-Fluorouracil Improves Outcomes in Cicatrizing Conjunctival Disorders Secondary to Systemic Disease".

Ophthalmic Plast Reconstr Surg 2022 Mar-Apr 01;38(2):208-210

Department of Ophthalmology, Oakland University William Beaumont School of Medicine, Rochester, MI.

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Re: "Direct Injection of 5-Fluorouracil Improves Outcomes in Cicatrizing Conjunctival Disorders Secondary to Systemic Disease".

Ophthalmic Plast Reconstr Surg 2022 Mar-Apr 01;38(2):208

Department of Oculoplasty and Ocular Oncology, Dr Shroff's Charity Eye Hospital, Daryaganj, New Delhi, India.

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Localized Blistering Eruption of the Face and Neck - A Case Study and Differential Considerations.

Clin Cosmet Investig Dermatol 2022 19;15:271-281. Epub 2022 Feb 19.

Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland.

We describe a 36-year-old woman with erythematous lesions and well-tense blisters confined to the face and neck of two months history, without mucosal involvement and no triggering factors. A lesional skin biopsy showed a subepidermal blister. Direct immunofluorescence of peribullous skin identified linear deposits of IgG, IgA, and C3 complement along the basement membrane zone, whereas indirect immunofluorescence was negative. Read More

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February 2022

Increased Fibrosis in a Mouse Model of Anti-Laminin 332 Mucous Membrane Pemphigoid Remains Unaltered by Inhibition of Aldehyde Dehydrogenase.

Front Immunol 2021 7;12:812627. Epub 2022 Feb 7.

Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the basal membrane zone of skin and surface-close epithelia and predominant mucosal lesions. The oral cavity and conjunctivae are most frequently affected, albeit clinical manifestations can also occur on the skin. MMP-associated lesions outside the oral cavity typically lead to scarring. Read More

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A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes.

F1000Res 2021 25;10:1201. Epub 2021 Nov 25.

The Cornea Institute, L V Prasad Eye Institute, Hyderabad, Telangana, India.

Ocular involvement in cases of bullous pemphigoid is rare and when present, the signs are usually subtle and in the form of fine tarsal scarring and dry eye disease. The current report aims to describe the clinical features and management protocols in a series of cases with aggressive ocular manifestations at presentation. All cases of bullous pemphigoid seen between 2017 and 2020 were included in this retrospective case series. Read More

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Desquamative gingivitis: a definite indicator for mucocutaneous disorders.

BMJ Case Rep 2022 Jan 17;15(1). Epub 2022 Jan 17.

Periodontics and Oral Implantology, Vydehi Institute of Dental Sciences, Bangalore, Karnataka, India.

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January 2022

Photobiomodulation of gingival lesions resulting from autoimmune diseases: systematic review and meta-analysis.

Clin Oral Investig 2022 May 13;26(5):3949-3964. Epub 2022 Jan 13.

School of Dentistry, Federal University of Alfenas, Rua Gabriel Monteiro da Silva, 700 - Centro, Alfenas, MG, 37130-001, Brazil.

Objectives: To evaluate the effects of photobiomodulation (PBM) in gingival lesions resulting from autoimmune diseases; to compare PBM and topical corticosteroid (CS) treatment; and to assess PBM outcome over time of follow-up.

Materials And Methods: A comprehensive electronic search was performed in four electronic databases. Treatment effects were measured through visual analog scale of pain (VAS) and clinical evolution of lesion (Thongprasom scale for oral lichen planus (OLP)). Read More

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Mucous membrane pemphigoid.

Autoimmun Rev 2022 Apr 4;21(4):103036. Epub 2022 Jan 4.

Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany; Department of Dermatology, Allergology and Venerology, University of Lübeck, Lübeck, Germany. Electronic address:

Mucous membrane pemphigoid (MMP) is a clinically and immunopathologically heterogenous disease with an incidence of about 2/million inhabitants/year in central Europe. Pemphigoid diseases are characterized by autoantibodies against structural proteins of the epidermis and/or surface-close epithelia. MMP has been defined as pemphigoid disease with predominant mucosal lesions. Read More

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Combination Therapy of Plasma Exchange and Rituximab to Treat Cicatricial Pemphigoid and Bullous Pemphigoid.

Cureus 2021 Nov 27;13(11):e19932. Epub 2021 Nov 27.

Dermatology, University of Central Florida College of Medicine, Orlando, USA.

The pemphigoid group of subepidermal autoimmune blistering diseases can affect both cutaneous and mucosal tissues. Therapy of this group of diseases, including cicatricial pemphigoid (CP) and bullous pemphigoid (BP), consists of systemic steroids and immunomodulatory agents. Recalcitrant cases have typically been treated with plasmapheresis or rituximab individually. Read More

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November 2021

Association Between Skin Findings and Ocular Signs in Rosacea.

Turk J Ophthalmol 2021 Dec;51(6):338-343

Hospital de Clínicas José de San Martin, Universidad de Buenos Aires (UBA), Department of Ophthalmology, Buenos Aires, Arjantin.

Objectives: To report the most frequent signs in ocular rosacea and evaluate their association with skin findings.

Materials And Methods: Fifty-one patients diagnosed with rosacea by a trained dermatologist were evaluated by an ocular surface specialist. A complete ophthalmological examination was performed. Read More

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December 2021

Corneal perforation in ocular cicatricial pemphigoid: A CARE-compliant case report.

Medicine (Baltimore) 2021 Dec;100(51):e28266

Department of Ophthalmology, Chi Mei Medical Center, Tainan, Taiwan.

Rationale: Ocular cicatricial pemphigoid (OCP) is a potentially blinding, rare systemic autoimmune disease. The definite etiology of OCP remains under debate, early diagnosis is important to prevent rapid deterioration. The majority of the discussion has been focused on its medical therapeutic strategy, while little effort has been made to study the role of amniotic membrane transplantation (AMT). Read More

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December 2021

Dermatologic management of ocular mucous membrane (cicatricial) pemphigoid with mycophenolate mofetil in 38 patients.

J Am Acad Dermatol 2021 Dec 17. Epub 2021 Dec 17.

Department of Dermatology, Center for Dermatology Research, Wake Forest School of Medicine, Winston-Salem, North Carolina.

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December 2021

Subunit-Specific Reactivity of Autoantibodies Against Laminin-332 Reveals Direct Inflammatory Mechanisms on Keratinocytes.

Front Immunol 2021 25;12:775412. Epub 2021 Nov 25.

Division of Dermatology, Rush University Medical Center, Chicago, IL, United States.

Laminin-332 pemphigoid is a rare and severe autoimmune blistering disease, caused by IgG autoantibodies targeting laminin-332 in the dermal-epidermal basement zone. Laminin-332 pemphigoid is characterized by variable inflammatory infiltrate and the predominance of non-complement-fixing antibodies. Given these findings, we hypothesized that IgG autoantibodies to laminin-332 directly resulted in keratinocyte expression of inflammatory factors. Read More

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February 2022

Comparison of Two Diagnostic Assays for Anti-Laminin 332 Mucous Membrane Pemphigoid.

Front Immunol 2021 25;12:773720. Epub 2021 Nov 25.

Center for Blistering Diseases, University of Groningen, Groningen, Netherlands.

Anti-laminin 332 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by predominant mucosal lesions and autoantibodies against laminin 332. The exact diagnosis of anti-laminin 332 MMP is important since nearly 30% of patients develop solid cancers. This study compared two independently developed diagnostic indirect immunofluorescence (IF) tests based on recombinant laminin 332 expressed in HEK239 cells (biochip mosaic assay) and the migration trails of cultured keratinocytes rich in laminin 332 (footprint assay). Read More

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February 2022

The Second Study of Clinical and Immunological Findings in Anti-laminin 332-Type Mucous Membrane Pemphigoid Examined at Kurume University-Diagnosis Criteria Suggested by Summary of 133 Cases.

Front Immunol 2021 26;12:771766. Epub 2021 Nov 26.

Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.

Background: Recently, we published an article retrospectively summarizing the results in 55 anti-laminin 332 (LM332)-type mucous membrane pemphigoid (MMP) cases examined at Kurume University, which were diagnosed by strict inclusion criteria, including positive reactivity in direct immunofluorescence and absence of antibodies to non-LM332 autoantigens. However, indirect immunofluorescence using 1M-NaCl-split normal human skin (ssIIF) is also valuable for diagnosis of anti-LM332-type MMP.

Methods: In this second study, we selected 133 anti-LM332-type MMP cases, which were diagnosed by our different inclusion criteria: (i) immunoglobulin G (IgG) deposition to basement membrane zone (BMZ) by direct immunofluorescence or IgG reactivity with dermal side of split skin by ssIIF, (ii) positivity for at least one of the three subunits of LM332 by immunoblotting of purified human LM332, and (iii) the presence of mucosal lesions. Read More

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February 2022

Insights into clinical and diagnostic findings as well as treatment responses in patients with mucous membrane pemphigoid: A retrospective cohort study.

J Am Acad Dermatol 2022 07 9;87(1):48-55. Epub 2021 Dec 9.

Department of Dermatology, Center of Blistering Diseases, Groningen, the Netherlands.

Background: The variable clinical severity of mucous membrane pemphigoid (MMP) often leads to diagnostic and therapeutic delays.

Objective: To describe the characteristics of a large cohort of patients with MMP.

Methods: A retrospective review of clinical and diagnostic characteristics as well as treatment responses in 145 patients with MMP. Read More

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Cicatricial pemphigoid Brunsting-Perry variant masquerading as neutrophil-mediated cicatricial alopecia.

J Cutan Pathol 2022 Apr 5;49(4):408-411. Epub 2022 Jan 5.

Department of Pathology, University of California, San Francisco, California, USA.

A 72-year-old male presented with scarring alopecia on the scalp vertex, multiple crusted plaques on the hairline, and a history of vesicular eruption on the face. The scalp showed crusted plaques with loss of follicular ostia. No follicular pustules or compound follicles were present. Read More

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