DOCSLIB.ORG

Pemphigus Foliaceus: a Case Report and Short Review

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Pemphigus Foliaceus: a Case Report and Short Review CONTINUING MEDICAL EDUCATION Pemphigus Foliaceus: A Case Report and Short Review Amor Khachemoune, MD, CWS; Kjetil Kristoffer Guldbakke, MD; Eric Ehrsam, MD GOAL To understand pemphigus foliaceus (PF) to better manage patients with the condition OBJECTIVES Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Distinguish between the various forms of pemphigus. 2. Recognize the clinical manifestations of PF. 3. Describe the treatment options for PF. CME Test on page 104. This article has been peer reviewed and approved Einstein College of Medicine is accredited by by Michael Fisher, MD, Professor of Medicine, the ACCME to provide continuing medical edu- Albert Einstein College of Medicine. Review date: cation for physicians. July 2006. Albert Einstein College of Medicine designates This activity has been planned and imple- this educational activity for a maximum of 1 AMA mented in accordance with the Essential Areas PRA Category 1 CreditTM. Physicians should only and Policies of the Accreditation Council for claim credit commensurate with the extent of their Continuing Medical Education through the participation in the activity. joint sponsorship of Albert Einstein College of This activity has been planned and produced in Medicine and Quadrant HealthCom, Inc. Albert accordance with ACCME Essentials. Drs. Khachemoune, Guldbakke, and Ehrsam report no conflict of interest. The authors discuss off-label use of azathioprine, corticosteroids, cyclophosphamide, cyclosporine, dapsone, extracorporeal photoche- motherapy, gold, hydroxychloroquine, intravenous immunoglobulin, methotrexate, mycophenolate mofetil, niacinamide, plasmapheresis, rituximab, and tetracycline. Dr. Fisher reports no conflict of interest. Pemphigus foliaceus (PF) is a rare autoimmune PF who was successfully treated with a combina- blistering disease presenting in endemic and tion of oral corticosteroids and dapsone. We also sporadic forms. The typical presentation is recur- provide a concise review of the literature and rent shallow erosions in a seborrheic distribution. discuss the etiology, clinical features, diagnosis, We present a case of a 58-year-old woman with and management of PF. Cutis. 2006;78:105-110. Accepted for publication January 6, 2006. Dr. Khachemoune is Assistant Professor, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, emphigus describes a group of autoimmune New York. Dr. Guldbakke currently is serving in the military in Norway. chronic bullous diseases, originally named Dr. Ehrsam is in private practice, Le Cateau, France. in 1791.1 The term pemphigus stems from the Reprints: Amor Khachemoune, MD, CWS, Ronald O. Perelman P 2 Department of Dermatology, New York University School of Greek pemphix meaning blister or bubble. Pemphi- Medicine, 530 First Ave, Suite 7R, New York, NY 10016 (e-mail: gus is usually divided into 2 major forms depending [email protected]). on blister location: pemphigus vulgaris (PV) and VOLUME 78, AUGUST 2006 105 Pemphigus Foliaceus pemphigus foliaceus (PF). Pemphigus vegetans is a variant of PV, and pemphigus erythematosus (PE) and fogo selvagem are variants of PF. During the past 3 decades, uncommon forms of pemphigus have been described, including pemphigus herpe- tiformis, immunoglobulin A (IgA) pemphigus, and paraneoplastic pemphigus.3 The incidence of pem- phigus ranges from 0.76 to 5 new cases per million per year.2 In a recent analysis of 1209 patients with pemphigus in Iran, the PV to PF ratio was found to be 12:1. However, this ratio varies widely in differ- ent parts of the world.4 In one report, 73% of cases of pemphigus in France were PV.5 PV also is the Superficial erosions on the chest. most common clinical type of pemphigus in Kuwait, Israel, and Singapore.6-8 In a report on pemphigus in South Africa, the most common clinical variant was for intermittent joint pain, but a literature search at PF, and 80% of these patients were black.9 Of note, that time did not reveal an association with similar in the Indian population in this region, PV was more eruptions; our patient was asked to discontinue the common. A high percentage of PF is endemic in celecoxib at the initiation of oral prednisone. She rural Brazil, Tunisia, and Columbia.9 was seen at regular follow-up visits every month. As the patient improved clinically, dapsone was Case Report reduced to 25 mg and finally discontinued after A 58-year-old white woman presented with a about 6 months. Oral prednisone taper continued 6-month history of superficial erosions on the slowly over a total of 18 months. No lesions were chest, upper back, hairline, and retroauricular present for the last 3 monthly visits. areas. She denied any photosensitivity, and there was no mucosal involvement. The patient’s medi- Comment cal history was otherwise unremarkable. Superficial PF comprises 2 major categories: endemic and spo- erosions with a positive Nikolsky sign were present radic. The endemic form, also known as fogo selvagem, on the chest (Figure), upper back, and frontal primarily affects children and young adults in rural hairline, as well as in the retroauricular areas bilat- Brazil. In contrast, the sporadic form of PF is gener- erally. Two punch biopsies (one lesional and one ally a disease of middle-aged individuals and the el- perilesional) were performed. Routine histology derly. Of note, there are several cases of nonendemic results showed intraepidermal vesicles in the upper PF occurring in children,10 and 2 cases reported granular layer containing acantholytic cells. Results in the neonatal period.11 Many patients with PE of direct immunofluorescence demonstrated IgG show serologic findings suggestive of systemic lupus and complement 3 (C3) in the intercellular spaces erythematosus (SLE), especially the presence of anti- in the epidermal cell surface. Of note, antinuclear nuclear antibodies.2,12 antibody and anti–double-stranded DNA were PF is an autoimmune blistering disease of negative. These findings were consistent with our unknown etiology with antibodies produced against clinical diagnosis of PF. desmoglein 1 (Dsg1).13 Dsg1 is an adhesive cadherin The patient was initially started on a high protein found in the desmosomal cell junction in the potency topical corticosteroid (clobetasol propi- suprabasal layers of the epidermis.14,15 Binding of the onate ointment) for 4 weeks with no improve- antibody results in the loss of cell adhesion or acan- ment. She was then given prednisone 60 mg/d for tholysis and formation of the clinical picture of PF.16 approximately one month when new lesions ceased The blisters are subcorneal, occurring in and around to appear; then the medication was slowly tapered the granular cell layer of the epidermis.13 Blister for- off. Dapsone also was added to her regimen after mation is superficial because the most differentiated an initial baseline level of glucose-6-phosphate layer of the epidermis is the only area in which Dsg1 dehydrogenase and a baseline complete blood is critically important to cell adhesion, and there is count were obtained. Dapsone was initiated at no protection redundancy of adhesion molecules by 50 mg/d while prednisone was being slowly tapered. coexpression of Dsg3.17 Of interest, one study has Cacit D3 in a single morning dose (1000 mg cal- demonstrated that the autoantibodies in up to 7% of cium and 880 IU vitamin D) also was added to the patients with PF and up to 50% of patients with PV regimen. Of note, the patient was taking celecoxib recognize both Dsg1 and Dsg3 isoforms.18 It also is 106 CUTIS® Pemphigus Foliaceus reported that some patients can progress from PF to show serologic findings suggestive of SLE, especially PV or vice versa, though the latter is less common.19 the presence of antinuclear antibodies.12,40 How- It has been shown that this transition correlates well ever, there are only a few reports of PE occurring with the qualitative and quantitative changes in in patients who have clearly defined SLE.41,42 In the profile of Dsg1 and Dsg3 antibodies.20 Patients most of these cases, the diagnosis of SLE had been with PF have been reported to have a predomi- established months to years earlier.42 PE also may be nance of circulating IgG4 antiepidermal autoantibod- associated with a variety of autoantibodies and may ies, followed by a lesser degree of IgG1, IgG2, and require extensive immunotherapy.43 PE has char- IgG3 subclasses.21 PF has shown a strong associa- acteristic findings on direct immunofluorescence, tion with several HLA-DRB1 haplotypes.22,23 Most usually IgG and C3 at the basement membrane recently, an association with HLA-DRB1*0101 was zone of erythematous facial skin, in addition to the found in the Mexican population.24 Sunlight expo- epidermal cell surface IgG.12,44 Clinically, the lesions sure25 and several drugs such as penicillamine26,27 of PE resemble those of PF but are most commonly have been identified as possible triggering factors for restricted to the upper trunk, head, and neck.42 PE the disease. In children, bacteria, cytomegalovirus, may remain localized for years, or it may evolve into and otitis also have been implicated.10 Similarly, an more generalized PF.40 PE also has been associated unusual case of childhood PF apparently triggered by with myasthenia gravis and thymomas.45 conjunctivitis was reported.28 The histologic changes of PF, PE, and fogo sel- PF manifests clinically as recurrent shallow ero- vagem are identical.2 Early blisters indicate acan- sions accompanied
Load more

Recommended publications
  • The Use of Biologic Agents in the Treatment of Oral Lesions Due to Pemphigus and Behçet's Disease: a Systematic Review
    Davis GE, Sarandev G, Vaughan AT, Al-Eryani K, Enciso R. The Use of Biologic Agents in the Treatment of Oral Lesions due to Pemphigus and Behçet’s Disease: A Systematic Review. J Anesthesiol & Pain Therapy. 2020;1(1):14-23 Systematic Review Open Access The Use of Biologic Agents in the Treatment of Oral Lesions due to Pemphigus and Behçet’s Disease: A Systematic Review Gerald E. Davis II1,2, George Sarandev1, Alexander T. Vaughan1, Kamal Al-Eryani3, Reyes Enciso4* 1Advanced graduate, Master of Science Program in Orofacial Pain and Oral Medicine, Herman Ostrow School of Dentistry of USC, Los Angeles, California, USA 2Assistant Dean of Academic Affairs, Assistant Professor, Restorative Dentistry, Meharry Medical College, School of Dentistry, Nashville, Tennessee, USA 3Assistant Professor of Clinical Dentistry, Division of Periodontology, Dental Hygiene & Diagnostic Sciences, Herman Ostrow School of Dentistry of USC, Los Angeles, California, USA 4Associate Professor (Instructional), Division of Dental Public Health and Pediatric Dentistry, Herman Ostrow School of Dentistry of USC, Los Angeles, California, USA Article Info Abstract Article Notes Background: Current treatments for pemphigus and Behçet’s disease, such Received: : March 11, 2019 as corticosteroids, have long-term serious adverse effects. Accepted: : April 29, 2020 Objective: The objective of this systematic review was to evaluate the *Correspondence: efficacy of biologic agents (biopharmaceuticals manufactured via a biological *Dr. Reyes Enciso, Associate Professor (Instructional), Division source) on the treatment of intraoral lesions associated with pemphigus and of Dental Public Health and Pediatric Dentistry, Herman Ostrow Behçet’s disease compared to glucocorticoids or placebo. School of Dentistry of USC, Los Angeles, California, USA; Email: [email protected].
    [Show full text]
  • The Neumann Type of Pemphigus Vegetans Treated with Combination of Dapsone and Steroid
    YM Son, et al Ann Dermatol Vol. 23, Suppl. 3, 2011 http://dx.doi.org/10.5021/ad.2011.23.S3.S310 CASE REPORT The Neumann Type of Pemphigus Vegetans Treated with Combination of Dapsone and Steroid Young-Min Son, M.D., Hong-Kyu Kang, M.D., Jeong-Hwan Yun, M.D., Joo-Young Roh, M.D., Jong-Rok Lee, M.D. Department of Dermatology, Gachon University of Medicine and Science, Gil Hospital, Incheon, Korea Pemphigus vegetans is a rare variant of pemphigus vulgaris INTRODUCTION and is characterized by vegetating lesions in the inguinal folds and mouth and by the presence of autoantibodies Pemphigus diseases are a group of autoimmune disorders against desmoglein 3. Two clinical subtypes of pemphigus that have certain common features, and these diseases are vegetans exist, which are initially characterized by flaccid considered to be potentially fatal1,2. Pemphigus vegetans bullae and erosions (the Neumann subtype) or pustules (the is a variant of pemphigus vulgaris and is the rarest form of Hallopeau subtype). Both subtypes subsequently develop pemphigus; Pemphigus vegetans comprises less than 1∼ into hyperpigmented vegetative plaques with pustules and 2% of all pemphigus cases1,3,4. This variant is charac- hypertrophic granulation tissue at the periphery of the terized by flaccid bullae or pustules that erode to form hy- lesions. Oral administration of corticosteroids alone does not pertrophic papillated plaques that predominantly involve always induce disease remission in patients with pemphigus the intertriginous areas, the scalp, and the face; in 60∼ vegetans. We report here on a 63-year-old woman with 80% of all cases, the oral mucosa are also affected5,6.
    [Show full text]
  • Vesiculobullous Diseases Larkin Community Hospital/NSU-COM Presenters: Yuri Kim, DO, Sam Ecker, DO, Jennifer David, DO, MBA
    Vesiculobullous Diseases Larkin Community Hospital/NSU-COM Presenters: Yuri Kim, DO, Sam Ecker, DO, Jennifer David, DO, MBA Program Director: Stanley Skopit, DO, MSE, FAOCD, FAAD •We have no relevant disclosures Topics of Discussion • Subcorneal Vesiculobullous Disorders – Pemphigus foliaceous – Pemphigus erythematosus – Subcorneal pustular dermatosis (Sneddon-Wilkinson Disease) – Acute Generalized Exanthematous Pustulosis • Intraepidermal Vesiculobullous Disorders – Pemphigus vulgaris – Pemphigus vegetans – Hailey-Hailey Disease – Darier’s Disease – Grover’s Disease – Paraneoplastic Pemphigus – IgA Pemphigus Topics of Discussion (Continued) • Pauci-inflammatory Subepidermal Vesiculobullous Disorders – Porphyria Cutanea Tarda (PCT) – Epidermolysis Bullosa Acquisita (EBA) – Pemphigoid Gestationis • Inflammatory Subepidermal Disorders – Bullous Pemphigoid – Cicatricial Pemphigoid – Dermatitis Herpetiformis – Linear IgA Subcorneal Vesiculobullous Disorders • Pemphigus foliaceous • Pemphigus erythematosus • Subcorneal pustular dermatosis (Sneddon- Wilkinson Disease) • AGEP Pemphigus Foliaceous • IgG Ab to desmoglein 1 (Dsg-1, 160 kDa) • Peak onset middle age, no gender preference • Endemic form – Fogo selvagem in Brazil and other parts of South America • Pemphigus erythematosus- Localized variant of pemphigus foliaceous with features of lupus erythematosus Overview Clinical H&E DIF Treatment Pemphigus Foliaceous Overview Clinical H&E DIF Treatment Pemphigus Foliaceous Overview Clinical H&E DIF Treatment Pemphigus Foliaceous Overview Clinical
    [Show full text]
  • Zeroing in on the Cause of Your Patient's Facial Pain
    Feras Ghazal, DDS; Mohammed Ahmad, Zeroing in on the cause MD; Hussein Elrawy, DDS; Tamer Said, MD Department of Oral Health of your patient's facial pain (Drs. Ghazal and Elrawy) and Department of Family Medicine/Geriatrics (Drs. Ahmad and Said), The overlapping characteristics of facial pain can make it MetroHealth Medical Center, Cleveland, Ohio difficult to pinpoint the cause. This article, with a handy at-a-glance table, can help. [email protected] The authors reported no potential conflict of interest relevant to this article. acial pain is a common complaint: Up to 22% of adults PracticE in the United States experience orofacial pain during recommendationS F any 6-month period.1 Yet this type of pain can be dif- › Advise patients who have a ficult to diagnose due to the many structures of the face and temporomandibular mouth, pain referral patterns, and insufficient diagnostic tools. disorder that in addition to Specifically, extraoral facial pain can be the result of tem- taking their medication as poromandibular disorders, neuropathic disorders, vascular prescribed, they should limit disorders, or atypical causes, whereas facial pain stemming activities that require moving their jaw, modify their diet, from inside the mouth can have a dental or nondental cause and minimize stress; they (FIGURE). Overlapping characteristics can make it difficult to may require physical therapy distinguish these disorders. To help you to better diagnose and and therapeutic exercises. C manage facial pain, we describe the most common causes and underlying pathological processes. › Consider prescribing a tricyclic antidepressant for patients with persistent idiopathic facial pain. C Extraoral facial pain Extraoral pain refers to the pain that occurs on the face out- 2-15 Strength of recommendation (SoR) side of the oral cavity.
    [Show full text]
  • Medicare Human Services (DHHS) Centers for Medicare & Coverage Issues Manual Medicaid Services (CMS) Transmittal 155 Date: MAY 1, 2002
    Department of Health & Medicare Human Services (DHHS) Centers for Medicare & Coverage Issues Manual Medicaid Services (CMS) Transmittal 155 Date: MAY 1, 2002 CHANGE REQUEST 2149 HEADER SECTION NUMBERS PAGES TO INSERT PAGES TO DELETE Table of Contents 2 1 45-30 - 45-31 2 2 NEW/REVISED MATERIAL--EFFECTIVE DATE: October 1, 2002 IMPLEMENTATION DATE: October 1, 2002 Section 45-31, Intravenous Immune Globulin’s (IVIg) for the Treatment of Autoimmune Mucocutaneous Blistering Diseases, is added to provide limited coverage for the use of IVIg for the treatment of biopsy-proven (1) Pemphigus Vulgaris, (2) Pemphigus Foliaceus, (3) Bullous Pemphigoid, (4) Mucous Membrane Pemphigoid (a.k.a., Cicatricial Pemphigoid), and (5) Epidermolysis Bullosa Acquisita. Use J1563 to bill for IVIg for the treatment of biopsy-proven (1) Pemphigus Vulgaris, (2) Pemphigus Foliaceus, (3) Bullous Pemphigoid, (4) Mucous Membrane Pemphigoid, and (5) Epidermolysis Bullosa Acquisita. This revision to the Coverage Issues Manual is a national coverage decision (NCD). The NCDs are binding on all Medicare carriers, intermediaries, peer review organizations, health maintenance organizations, competitive medical plans, and health care prepayment plans. Under 42 CFR 422.256(b), an NCD that expands coverage is also binding on a Medicare+Choice Organization. In addition, an administrative law judge may not review an NCD. (See §1869(f)(1)(A)(i) of the Social Security Act.) These instructions should be implemented within your current operating budget. DISCLAIMER: The revision date and transmittal number only apply to the redlined material. All other material was previously published in the manual and is only being reprinted. CMS-Pub.
    [Show full text]
  • Oral Manifestations of Systemic Disease Their Clinical Practice
    ARTICLE Oral manifestations of systemic disease ©corbac40/iStock/Getty Plus Images S. R. Porter,1 V. Mercadente2 and S. Fedele3 provide a succinct review of oral mucosal and salivary gland disorders that may arise as a consequence of systemic disease. While the majority of disorders of the mouth are centred upon the focus of therapy; and/or 3) the dominant cause of a lessening of the direct action of plaque, the oral tissues can be subject to change affected person’s quality of life. The oral features that an oral healthcare or damage as a consequence of disease that predominantly affects provider may witness will often be dependent upon the nature of other body systems. Such oral manifestations of systemic disease their clinical practice. For example, specialists of paediatric dentistry can be highly variable in both frequency and presentation. As and orthodontics are likely to encounter the oral features of patients lifespan increases and medical care becomes ever more complex with congenital disease while those specialties allied to disease of and effective it is likely that the numbers of individuals with adulthood may see manifestations of infectious, immunologically- oral manifestations of systemic disease will continue to rise. mediated or malignant disease. The present article aims to provide This article provides a succinct review of oral manifestations a succinct review of the oral manifestations of systemic disease of of systemic disease. It focuses upon oral mucosal and salivary patients likely to attend oral medicine services. The review will focus gland disorders that may arise as a consequence of systemic upon disorders affecting the oral mucosa and salivary glands – as disease.
    [Show full text]
  • Paraneoplastic Pemphigus with Clinical Features of Lichen Planus Associated with Low-Grade B Cell Lymphoma
    Report Paraneoplastic pemphigus with clinical features of lichen planus associated with low-grade B cell lymphoma Sónia Coelho, MD, José Pedro Reis, MD, Oscar Tellechea, MD, PhD, Américo Figueiredo, MD, PhD, and Martin Black, MD, PhD From the Department of Dermatology, Abstract University Hospital, Coimbra, Portugal, St Background Neoplasia-induced lichen planus is described as a cell-mediated reaction to John’s Institute of Dermatology, St Thomas’ unknown epithelial antigens. Paraneoplastic pemphigus (PNP), characterized by the presence Hospital, London, UK of a specific array of autoantibodies, probably represents a different form of presentation of the Correspondence same autoimmune syndrome where the mucocutaneous expression depends on the dominant Sónia Coelho pathologic mechanism. Clínica de Dermatologia, Hospital da Methods The authors report a case of PNP with predominant lichen planus-like lesions and Universidade review the relevant literature. We observed a 74-year-old female with vesico-bullous, erosive, P.3000–075 Coimbra target-shaped and flat papular lichenoid lesions on the lower legs, palms and soles, evolving for Portugal E-mail: [email protected] 3 weeks. Histopathology revealed a lichenoid dermatitis. Direct immunofluorescence showed C3 deposition around keratinocytes and epidermal IgG intranuclear deposition. Indirect immunofluorescence revealed circulating IgG with intercellular staining on rat bladder substrate. Immunoblotting demonstrated bands of 130, 190, 210 and 250 kDa antigens. A pararenal B cell lymphoma was found. Results Oral corticotherapy with 40 mg prednisolone daily was initiated with a good cutaneous response. Four months later, cyclophosphamide (50 mg/day) was introduced because of a discrete enlargement of the pararenal mass. The patient died on the seventh month of follow up as a result of respiratory insufficiency.
    [Show full text]
  • Cardiovascular Drugs-Induced Oral Toxicities: a Murky Area to Be Revisited and Illuminated
    Pharmacological Research 102 (2015) 81–89 Contents lists available at ScienceDirect Pharmacological Research j ournal homepage: www.elsevier.com/locate/yphrs Review Cardiovascular drugs-induced oral toxicities: A murky area to be revisited and illuminated a, b b Pitchai Balakumar ∗, Muthu Kavitha , Suresh Nanditha a Pharmacology Unit, Faculty of Pharmacy, AIMST University, Semeling, 08100 Bedong, Malaysia b Faculty of Dentistry, AIMST University, 08100 Bedong, Malaysia a r t i c l e i n f o a b s t r a c t Article history: Oral health is an imperative part of overall human health. Oral disorders are often unreported, but are Received 20 July 2015 highly troublesome to human health in a long-standing situation. A strong association exists between Received in revised form 22 August 2015 cardiovascular drugs and oral adverse effects. Indeed, several cardiovascular drugs employed clinically Accepted 8 September 2015 have been reported to cause oral adverse effects such as xerostomia, oral lichen planus, angioedema, Available online 25 September 2015 aphthae, dysgeusia, gingival enlargement, scalded mouth syndrome, cheilitis, glossitis and so forth. Oral complications might in turn worsen the cardiovascular disease condition as some reports suggest an Keywords: adverse correlation between periodontal oral disease pathogenesis and cardiovascular disease. These are Cardiovascular drugs certainly important to be understood for a better use of cardiovascular medicines and control of associated Oral adverse effects oral adverse effects. This review sheds lights on the oral adverse effects pertaining to the clinical use of Dry mouth Angioedema cardiovascular drugs. Above and beyond, an adverse correlation between oral disease and cardiovascular Dysgeusia disease has been discussed.
    [Show full text]
  • Pemphigus. S2 Guideline for Diagnosis and Treatment
    DOI: 10.1111/jdv.12772 JEADV GUIDELINES Pemphigus. S2 Guideline for diagnosis and treatment – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV) M. Hertl,1,* H. Jedlickova,2 S. Karpati,3 B. Marinovic,4 S. Uzun,5 S. Yayli,6 D. Mimouni,7 L. Borradori,8 C. Feliciani,9 D. Ioannides,10 P. Joly,11 C. Kowalewski,12 G. Zambruno,13 D. Zillikens,14 M.F. Jonkman15 1Department of Dermatology, Philipps-University Marburg, Marburg, Germany 2Department of Dermatology, Masaryk University, Brno, Czech Republic 3Department of Dermatology, Semmelweis University Budapest, Budapest, Hungary 4Department of Dermatology, School of Medicine University of Zagreb, Zagreb, Croatia 5Department of Dermatology, Akdeniz University, Antalya, Turkey 6Department of Dermatology, Karadeniz Technical University, Trabzon, Turkey 7Department of Dermatology, Tel-Aviv University, Tel-Aviv, Israel 8Department of Dermatology, University of Bern, Inselspital, Switzerland 9Department of Dermatology, University of Parma, Parma, Italy 10Department of Dermatology, Aristotle University of Thessaloniki, Thessaloniki, Greece 11Department of Dermatology, Rouen University Hospital, Rouen, France 12Department of Dermatology, Medical University of Warsaw, Warsaw, Poland 13Department of Dermatology, L’Istituto Dermopatico dell’Immacolata, Rome, Italy 14Department of Dermatology, University of Lubeck,€ Lubeck,€ Germany 15Department of Dermatology, University of Groningen, Groningen, The Netherlands *Correspondence: M. Hertl. E-mail: [email protected] Abstract Background Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blis- ters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost fatal. Due to its rarity, only few prospective controlled therapeutic trials are available.
    [Show full text]
  • Pemphigus Foliaceus Igg Causes Dissociation of Desmoglein 1– Containing Junctions Without Blocking Desmoglein 1 Transinteraction
    Pemphigus foliaceus IgG causes dissociation of desmoglein 1– containing junctions without blocking desmoglein 1 transinteraction Jens Waschke, … , Detlef Zillikens, Detlev Drenckhahn J Clin Invest. 2005;115(11):3157-3165. https://doi.org/10.1172/JCI23475. Research Article Dermatology Autoantibodies against the epidermal desmosomal cadherins desmoglein 1 (Dsg1) and Dsg3 have been shown to cause severe to lethal skin blistering clinically defined as pemphigus foliaceus (PF) and pemphigus vulgaris (PV). It is unknown whether antibody-induced dissociation of keratinocytes is caused by direct inhibition of Dsg1 transinteraction or by secondary cellular responses. Here we show in an in vitro system that IgGs purified from PF patient sera caused cellular dissociation of cultured human keratinocytes as well as significant release of Dsg1-coated microbeads attached to Dsg- containing sites on the keratinocyte cellular surface. However, cell dissociation and bead release induced by PF-IgGs was not caused by direct steric hindrance of Dsg1 transinteraction, as demonstrated by single molecule atomic force measurements and by laser trapping of surface-bound Dsg1-coated microbeads. Rather, our experiments strongly indicate that PF-IgG–mediated dissociation events must involve autoantibody-triggered cellular signaling pathways, resulting in destabilization of Dsg1-based adhesive sites and desmosomes. Find the latest version: https://jci.me/23475/pdf Research article Pemphigus foliaceus IgG causes dissociation of desmoglein 1–containing junctions without blocking desmoglein 1 transinteraction Jens Waschke,1 Paola Bruggeman,1 Werner Baumgartner,1 Detlef Zillikens,2 and Detlev Drenckhahn1 1Institute of Anatomy and Cell Biology, University of Würzburg, Würzburg, Germany. 2Department of Dermatology, University of Lübeck, Lübeck, Germany. Autoantibodies against the epidermal desmosomal cadherins desmoglein 1 (Dsg1) and Dsg3 have been shown to cause severe to lethal skin blistering clinically defined as pemphigus foliaceus (PF) and pemphigus vulgaris (PV).
    [Show full text]
  • A Case of Pemphigus Vegetans Presenting with Hoarseness of Voice
    AUTOIMMUNE BULLOUS DISEASES A CASE OF PEMPHIGUS VEGETANS PRESENTING WITH HOARSENESS OF VOICE H Kaur (1) - Mmq Liau (2) - Xq Koh (2) - J Huang (3) - Cl Tan (2) Yong Loo Lin School Of Medicine, National University Of Singapore, Singapore, Singapore (1) - Department Of Dermatology, National University Hospital, Singapore, Singapore (2) - Department Of Pathology, National University Hospital, Singapore, Singapore (3) Background: Pemphigus vegetans is a rare clinical variant of pemphigus vulgaris. It is characterised by flaccid blisters, erosions, vegetative proliferations and involvement of mucous membranes in particular the oral cavity. Laryngeal involvement has been infrequently reported and poorly characterised. Observation: We report a case of pemphigus vegetans in an 89-year-old Chinese gentlemen who presented with a 2-week history of hoarseness of voice. On examination, there were extensive haemorrhagic ulcers and erosions over the hard and soft palate, buccal mucosa, upper and lower lips. These were accompanied by multiple vegetative plaques scattered over his scrotum and perineum. Nasoendoscopy examination revealed features of vocal cord webbing and evidence of chronic inflammatory mucosal disease. Antibodies to desmoglein 3 was positive and histological examination was consistent with pemphigus vegetans. Key message: Laryngeal involvement in pemphigus vegetans have been poorly characterised in the literature. Therefore, it may not be immediately intuitive for physicians to associate the condition with patients presenting with symptoms such as hoarseness of voice. This unique presentation of pemphigus illustrated in our case is a reminder to consider the diverse presentations of this condition. In addition, it is important to rule out differential diagnosis such as extra-intestinal manifestations of Crohn’s disease, recurrent aphthous stomatitis, pyostomatitis vegetans, mucous membrane pemphigoid and HSV gingivostomatitis.
    [Show full text]
  • Clinical PRACTICE Blistering Mucocutaneous Diseases of the Oral Mucosa — a Review: Part 2
    Clinical PRACTICE Blistering Mucocutaneous Diseases of the Oral Mucosa — A Review: Part 2. Pemphigus Vulgaris Contact Author Mark R. Darling, MSc (Dent), MSc (Med), MChD (Oral Path); Dr.Darling Tom Daley, DDS, MSc, FRCD(C) Email: mark.darling@schulich. uwo.ca ABSTRACT Oral mucous membranes may be affected by a variety of blistering mucocutaneous diseases. In this paper, we review the clinical manifestations, typical microscopic and immunofluorescence features, pathogenesis, biological behaviour and treatment of pemphigus vulgaris. Although pemphigus vulgaris is not a common disease of the oral cavity, its potential to cause severe or life-threatening disease is such that the general dentist must have an understanding of its pathophysiology, clinical presentation and management. © J Can Dent Assoc 2006; 72(1):63–6 MeSH Key Words: mouth diseases; pemphigus/drug therapy; pemphigus/etiology This article has been peer reviewed. he most common blistering conditions captopril, phenacetin, furosemide, penicillin, of the oral and perioral soft tissues were tiopronin and sulfones such as dapsone. Oral Tbriefly reviewed in part 1 of this paper lesions are commonly seen with pemphigus (viral infections, immunopathogenic mucocu- vulgaris and paraneoplastic pemphigus.6 taneous blistering diseases, erythema multi- forme and other contact or systemic allergic Normal Desmosomes reactions).1–4 This paper (part 2) focuses on Adjacent epithelial cells share a number of the second most common chronic immuno- connections including tight junctions, gap pathogenic disease to cause chronic oral junctions and desmosomes. Desmosomes are blistering: pemphigus vulgaris. specialized structures that can be thought of as spot welds between cells. The intermediate Pemphigus keratin filaments of each cell are linked to focal Pemphigus is a group of diseases associated plaque-like electron dense thickenings on the with intraepithelial blistering.5 Pemphigus inside of the cell membrane containing pro- vulgaris (variant: pemphigus vegetans) and teins called plakoglobins and desmoplakins.
    [Show full text]