The Neumann Type of Pemphigus Vegetans Treated with Combination of Dapsone and Steroid
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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 -
2017 Oregon Dental Conference® Course Handout
2017 Oregon Dental Conference® Course Handout Nasser Said-Al-Naief, DDS, MS Course 8125: “The Mouth as The Body’s Mirror: Oral, Maxillofacial, and Head and Neck Manifestations of Systemic Disease” Thursday, April 6 2 pm - 3:30 pm 2/28/2017 The Mouth as The Body’s Mirror Oral Maxillofacial and Head and Neck Manifestation of Ulcerative Conditions of Allergic & Immunological Systemic Disease the Oro-Maxillofacial Diseases Region Nasser Said-Al-Naief, DDS, MS Professor & Chair, Oral Pathology and Radiology Director, OMFP Laboratory Oral manifestations of Office 503-494-8904// Direct: 503-494-0041 systemic diseases Oral Manifestations of Fax: 503-494-8905 Dermatological Diseases Cell: 1-205-215-5699 Common Oral [email protected] Conditions [email protected] OHSU School of Dentistry OHSU School of Medicine 2730 SW Moody Ave, CLSB 5N008 Portland, Oregon 97201 Recurrent aphthous stomatitis (RAS) Recurrent aphthous stomatitis (RAS) • Aphthous" comes from the Greek word "aphtha”- • Recurrence of one or more painful oral ulcers, in periods of days months. = ulcer • Usually begins in childhood or adolescence, • The most common oral mucosal disease in North • May decrease in frequency and severity by age America. (30+). • Affect 5% to 66% of the North American • Ulcers are confined to the lining (non-keratinized) population. mucosa: • * 60% of those affected are members of the • Buccal/labial mucosa, lateral/ventral tongue/floor of professional class. the mouth, soft palate/oropharyngeal mucosa • Etiopathogenesis: 1 2/28/2017 Etiology of RAU Recurrent Aphthous Stomatitis (RAS): Types: Minor; small size, shallow, regular, preceeded by prodrome, heal in 7-10 days Bacteria ( S. -
Orofacial Granulomatosis
Al-Hamad, A; Porter, S; Fedele, S; (2015) Orofacial Granulomatosis. Dermatol Clin , 33 (3) pp. 433- 446. 10.1016/j.det.2015.03.008. Downloaded from UCL Discovery: http://discovery.ucl.ac.uk/1470143 ARTICLE Oro-facial Granulomatosis Arwa Al-Hamad1, 2, Stephen Porter1, Stefano Fedele1, 3 1 University College London, UCL Eastman Dental Institute, Oral Medicine Unit, 256 Gray’s Inn Road, WC1X 8LD, London UK. 2 Dental Services, King Abdulaziz Medical City-Riyadh, Ministry of National Guard, Riyadh, Saudi Arabia. 3 NIHR University College London Hospitals Biomedical Research Centre, London, UK. Acknowledgments: Part of this work was undertaken at University College London/University College London Hospital, which received a proportion of funding from the Department of Health’s National Institute for Health Research Biomedical Research Centre funding scheme. Conflicts of Interest: The authors declare that they have no affiliation with any organization with a financial interest, direct or indirect, in the subject matter or materials discussed in the manuscript that may affect the conduct or reporting of the work submitted. Authorship: all authors named above meet the following criteria of the International Committee of Medical Journal Editors: 1) Substantial contributions to conception and design, or acquisition of data, or analysis and interpretation of data; 2) Drafting the article or revising it critically for important intellectual content; 3) Final approval of the version to be published. Corresponding author: Dr. Stefano Fedele DDS, PhD -
Oral Pathology Unmasking Gastrointestinal Disease
Journal of Dental Health Oral Disorders & Therapy Review Article Open Access Oral pathology unmasking gastrointestinal disease Abstract Volume 5 Issue 5 - 2016 Different ggastrointestinal disorders, such as Gastroesophageal Reflux Disease (GERD), Celiac Disease (CD) and Crohn’s disease, may manifest with alterations of the oral cavity Fumagalli LA, Gatti H, Armano C, Caruggi S, but are often under and misdiagnosed both by physicians and dentists. GERD can cause Salvatore S dental erosions, which are the main oral manifestation of this disease, or other multiple Department of Pediatric, Università dell’Insubria, Italy affections involving both hard and soft tissues such as burning mouth, aphtous oral ulcers, Correspondence: Silvia Salvatore, Pediatric Department of erythema of soft palate and uvula, stomatitis, epithelial atrophy, increased fibroblast number Pediatric, Università dell’Insubria, Via F. Del Ponte 19, 21100 in chorion, xerostomia and drooling. CD may be responsible of recurrent aphthous stomatitis Varese, Italy, Tel 0039 0332 299247, Fax 0039 0332 235904, (RAS), dental enamel defects, delayed eruption of teeth, atrophic glossitis and angular Email chelitis. Crohn’s disease can occur with several oral manifestations like indurated tag-like lesions, clobbestoning, mucogingivitis or, less specifically, with RAS, angular cheilitis, Received: October 30, 2016 | Published: December 12, 2016 reduced salivation, halitosis, dental caries and periodontal involvement, candidiasis, odynophagia, minor salivary gland enlargement, perioral -
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. -
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. -
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. -
SKIN VERSUS PEMPHIGUS FOLIACEUS and the AUTOIMMUNE GANG Lara Luke, BS, RVT, Dermatology, Purdue Veterinary Teaching Hospital
VETERINARY NURSING EDUCATION SKIN VERSUS PEMPHIGUS FOLIACEUS AND THE AUTOIMMUNE GANG Lara Luke, BS, RVT, Dermatology, Purdue Veterinary Teaching Hospital This program was reviewed and approved by the AAVSB Learning Objective: After reading this article, the participant will be able to dis- RACE program for 1 hour of continuing education in jurisdictions which recognize AAVSB RACE approval. cuss and compare autoimmune diseases that have dermatological afects, includ- Please contact the AAVSB RACE program if you have any ing Pemphigus Foliaceus (PF), Pemphigus Erythematosus (PE), Discoid Lupus comments/concerns regarding this program’s validity or relevancy to the veterinary profession. Erythematosus (DLE), Systemic Lupus Erythematosus (SLE). In addition, the reader will become familiar with diagnostic and treatment techniques. FUNCTION OF THE SKIN Te skin is the largest organ of the body. Along with sensory function, it provides a barrier between the inside and outside world. Te epidermis is composed of the following fve layers: stratum basale, stratum spinosum, stratum granulosum, stratum lucidum, and stratum corneum. Te stratum lucidum is found only on the nasal planum and footpads. When the cells of the epidermis are disrupted by systemic disease, the barrier is also disrupted. Clinical signs of skin disease will bring the patient into the veterinarian’s ofce for diagnosis. 32 THE NAVTA JOURNAL | NAVTA.NET VETERINARY NURSING EDUCATION THE PEMPHIGUS COMPLEX article.3 Histologically it shares characteris- Pemphigus Foliaceus tics of both PF and DLE.1 This classifcation PF is an immune mediated pustular disor- is still considered controversial and PE may der included in a group of diseases known just be a localized variant of PF.1 as the pemphigus complex. -
Oral Ulcers Presentation in Systemic Diseases: an Update
Open Access Maced J Med Sci electronic publication ahead of print, published on October 10, 2019 as https://doi.org/10.3889/oamjms.2019.689 ID Design Press, Skopje, Republic of Macedonia Open Access Macedonian Journal of Medical Sciences. https://doi.org/10.3889/oamjms.2019.689 eISSN: 1857-9655 Review Article Oral Ulcers Presentation in Systemic Diseases: An Update Sadia Minhas1, Aneequa Sajjad1, Muhammad Kashif2, Farooq Taj3, Hamed Al Waddani4, Zohaib Khurshid5* 1Department of Oral Pathology, Akhtar Saeed Dental College, Lahore, Pakistan; 2Department of Oral Pathology, Bakhtawar Amin Medical & Dental College, Multan, Pakistan; 3Department of Prosthetic, Khyber Medical University Institute of Dental Sciences, Kohat, Pakistan; 4Department of Medicine and Surgery, College of Dentistry, King Faisal University, Hofuf, Al- Ahsa Governorate, Saudi Arabia; 5Department of Prosthodontics and Dental Implantology, College of Dentistry, King Faisal University, Hofuf, Al-Ahsa Governorate, Saudi Arabia Abstract Citation: Minhas S, Sajjad A, Kashif M, Taj F, Al BACKGROUND: Diagnosis of oral ulceration is always challenging and has been the source of difficulty because Waddani H, Khurshid Z. Open Access Maced J Med Sci. of the remarkable overlap in their clinical presentations. https://doi.org/10.3889/oamjms.2019.689 Keywords: Oral ulcer; Infections; Vesiculobullous lesion; AIM: The objective of this review article is to provide updated knowledge and systemic approach regarding oral Traumatic ulcer; Systematic disease ulcers diagnosis depending upon clinical picture while excluding the other causative causes. *Correspondence: Zohaib Khurshid. Department of Prosthodontics and Dental Implantology, College of Dentistry, King Faisal University, Hofuf, Al-Ahsa METHODS: For this, specialised databases and search engines involving Science Direct, Medline Plus, Scopus, Governorate, Saudi Arabia. -
Orofacial Granulomatosis
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by UCL Discovery Al-Hamad, A; Porter, S; Fedele, S; (2015) Orofacial Granulomatosis. Dermatol Clin , 33 (3) pp. 433- 446. 10.1016/j.det.2015.03.008. Downloaded from UCL Discovery: http://discovery.ucl.ac.uk/1470143 ARTICLE Oro-facial Granulomatosis Arwa Al-Hamad1, 2, Stephen Porter1, Stefano Fedele1, 3 1 University College London, UCL Eastman Dental Institute, Oral Medicine Unit, 256 Gray’s Inn Road, WC1X 8LD, London UK. 2 Dental Services, King Abdulaziz Medical City-Riyadh, Ministry of National Guard, Riyadh, Saudi Arabia. 3 NIHR University College London Hospitals Biomedical Research Centre, London, UK. Acknowledgments: Part of this work was undertaken at University College London/University College London Hospital, which received a proportion of funding from the Department of Health’s National Institute for Health Research Biomedical Research Centre funding scheme. Conflicts of Interest: The authors declare that they have no affiliation with any organization with a financial interest, direct or indirect, in the subject matter or materials discussed in the manuscript that may affect the conduct or reporting of the work submitted. Authorship: all authors named above meet the following criteria of the International Committee of Medical Journal Editors: 1) Substantial contributions to conception and design, or acquisition of data, or analysis and interpretation of data; 2) Drafting the article or revising it critically for important intellectual content; -
Skin Brief Articles
SKIN BRIEF ARTICLES An Atypical Presentation of Pemphigus Vegetans in the Umbilicus Antonio Jimenez, BS1, Paige Hoyer, MD2, Michael Wilkerson, MD2 1The University of Texas Medical Branch, School of Medicine, Galveston, TX 2The University of Texas Medical Branch, Department of Dermatology, Galveston, TX ABSTRACT Pemphigus is a chronic, autoimmune bullous disease that affects the skin and mucous membranes. Pemphigus vegetans is a rare variant of pemphigus and presents as oral ulcerations with associated verrucous lesions in intertriginous or flexural areas. A 38-year-old African American woman presented to the clinic with a chief complaint of oral ulcers. She carried a diagnosis of Behcet’s disease and was referred by rheumatology for evaluation of treatment- resistant mucosal ulcerations. At the time of her dermatology visit, she also reported an enlarging umbilical mass that had been present for several months. Further examination of the umbilical lesion identified an exophytic, vegetative mass. Histologic assessment of the lesion identified acanthosis and acantholysis with dermal eosinophils consistent with pemphigus vegetans. A pemphigus antibody panel was done and resulted positive for IgG desmoglein-3 antibodies. The patient was treated with prednisone and rituximab with improvement of her lesions. We present an atypical presentation of pemphigus vegetans involving the umbilicus. This diagnosis should be considered in patients who present with oral erosions and concomitant vegetative lesions, regardless of location or prior diagnoses. pemphigus vulgaris, it most often presents INTRODUCTION with verrucous plaques in intertriginous and flexural areas. The rare presentation of the The word “pemphigus” is derived from the disease makes it a diagnostic challenge. We Greek word “pemphix” or blister.1 present a case of pemphigus vegetans in Pemphigus is a rare, blistering the umbilicus of a 38-year-old female. -
Pemphigus and Other Bullous Dermatoses: Correlation of Clinical and Pathologic Findings
View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector PEMPHIG[JS AND OTHER BULLOUS DERMATOSES: CORRELATION OF CLINICAL AND PATHOLOGIC FINDINGS" JOSEPH G. BRENNAN, M.D.t AND HAMILTON MONTGOMERY, M.D4 A detailed clinical and histopathologic study has been made of patients who had pemphigus of various types and of patients who had other bullous derma- toses; this study includes follow-up information covering a period of years. The lesions in some of these patients have had to he reclassified on the basis of newer histologic concepts of pemphigus, namely the phenomenon of acantholysis, which is seen in pemphigus and not in other bullous dermatoses, except for those caused by viral diseases. By acantholysis is meant loss of prickles of the prickle cells in the epidermis, with lysis of the individual cell. This phenomenon, occurring as a primary change in pemphigus in distinction from pressure or tension phenomena, which are the primary changes in other bullous dermatoses (except for pemphi- gus and viral diseases), is considered in another article by one of us (Brennan, 1). Differentiation of bullous dermatoses was accepted clinically prior to 1848, when Simon (2) considered that the bulla of pemphigus could be differentiated histologically because it was subepidermal and because the hair follicle was torn off. Haight (3), in 1868, thought the bulla of pemphigus was under the stratum corneum and was characterized by fissures formed by long, drawn-out prickle cells. Auspitz (4), in 1881, classified bullous dermatoses accord- ing to their histology and was the first to describe acantholysis.