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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. -
Understanding and Managing Scleroderma
Understanding and Managing Scleroderma A publication of Scleroderma Foundation 300 Rosewood Drive, Suite 105 Danvers, MA 01923 Maureen D. Mayes, M.D., M.P.H. Understanding and Understanding My notes and Managing Scleroderma Managing Scleroderma This booklet is intended to help people with scleroderma, their families and others interested ________________________ in learning more about the disease to better understand what scleroderma is, what effects ________________________ it may have, and what those with scleroderma can do to help themselves and their physicians ________________________ manage the disease. It answers some of the most frequently asked questions about ________________________ A publication of Maureen D. Mayes, M.D., M.P.H. Scleroderma Foundation 300 Rosewood Drive, Suite 105 scleroderma. Danvers, MA 01923 800-722-HOPE (4673) www.scleroderma.org www.facebook.com/sclerodermaUS www.twitter.com/scleroderma ________________________ Disclaimer The Scleroderma Foundation does not provide medical advice nor does it ________________________ endorse any drug or treatment mentioned herein. ________________________ The material contained in this booklet is presented for general information only. It is not intended to provide medical advice, to answer questions specific to the condition or problems of particular individuals, nor in ________________________ any way to substitute for the professional advice and care of qualified physicians. Mention of particular drugs and/or treatments is for ________________________ information purposes only and does not constitute an endorsement of said drugs and/or treatments. ________________________ Thanks! ________________________ The Scleroderma Foundation expresses its deep appreciation to the many ________________________ physicians whose efforts have led to this booklet. Special thanks are owed to Maureen D. Mayes, M.D., M.P.H., of the ________________________ University of Texas McGovern Medical School, Houston. -
Dermatological Findings in Common Rheumatologic Diseases in Children
Available online at www.medicinescience.org Medicine Science ORIGINAL RESEARCH International Medical Journal Medicine Science 2019; ( ): Dermatological findings in common rheumatologic diseases in children 1Melike Kibar Ozturk ORCID:0000-0002-5757-8247 1Ilkin Zindanci ORCID:0000-0003-4354-9899 2Betul Sozeri ORCID:0000-0003-0358-6409 1Umraniye Training and Research Hospital, Department of Dermatology, Istanbul, Turkey. 2Umraniye Training and Research Hospital, Department of Child Rheumatology, Istanbul, Turkey Received 01 November 2018; Accepted 19 November 2018 Available online 21.01.2019 with doi:10.5455/medscience.2018.07.8966 Copyright © 2019 by authors and Medicine Science Publishing Inc. Abstract The aim of this study is to outline the common dermatological findings in pediatric rheumatologic diseases. A total of 45 patients, nineteen with juvenile idiopathic arthritis (JIA), eight with Familial Mediterranean Fever (FMF), six with scleroderma (SSc), seven with systemic lupus erythematosus (SLE), and five with dermatomyositis (DM) were included. Control group for JIA consisted of randomly chosen 19 healthy subjects of the same age and gender. The age, sex, duration of disease, site and type of lesions on skin, nails and scalp and systemic drug use were recorded. χ2 test was used. The most common skin findings in patients with psoriatic JIA were flexural psoriatic lesions, the most common nail findings were periungual desquamation and distal onycholysis, while the most common scalp findings were erythema and scaling. The most common skin finding in patients with oligoarthritis was photosensitivity, while the most common nail finding was periungual erythema, and the most common scalp findings were erythema and scaling. We saw urticarial rash, dermatographism, nail pitting and telogen effluvium in one patient with systemic arthritis; and photosensitivity, livedo reticularis and periungual erythema in another patient with RF-negative polyarthritis. -
Visual Recognition of Autoimmune Connective Tissue Diseases
Seeing the Signs: Visual Recognition of Autoimmune Connective Tissue Diseases Utah Association of Family Practitioners CME Meeting at Snowbird, UT 1:00-1:30 pm, Saturday, February 13, 2016 Snowbird/Alta Rick Sontheimer, M.D. Professor of Dermatology Univ. of Utah School of Medicine Potential Conflicts of Interest 2016 • Consultant • Paid speaker – Centocor (Remicade- – Winthrop (Sanofi) infliximab) • Plaquenil – Genentech (Raptiva- (hydroxychloroquine) efalizumab) – Amgen (etanercept-Enbrel) – Alexion (eculizumab) – Connetics/Stiefel – MediQuest • Royalties Therapeutics – Lippincott, – P&G (ChelaDerm) Williams – Celgene* & Wilkins* – Sanofi/Biogen* – Clearview Health* Partners • 3Gen – Research partner *Active within past 5 years Learning Objectives • Compare and contrast the presenting and Hallmark cutaneous manifestations of lupus erythematosus and dermatomyositis • Compare and contrast the presenting and Hallmark cutaneous manifestations of morphea and systemic sclerosis Distinguishing the Cutaneous Manifestations of LE and DM Skin involvement is 2nd most prevalent clinical manifestation of SLE and 2nd most common presenting clinical manifestation Comprehensive List of Skin Lesions Associated with LE LE-SPECIFIC LE-NONSPECIFIC Cutaneous vascular disease Acute Cutaneous LE Vasculitis Leukocytoclastic Localized ACLE Palpable purpura Urticarial vasculitis Generalized ACLE Periarteritis nodosa-like Ten-like ACLE Vasculopathy Dego's disease-like Subacute Cutaneous LE Atrophy blanche-like Periungual telangiectasia Annular Livedo reticularis -
ACTINIDIACEAE 1. ACTINIDIA Lindley, Nat. Syst. Bot., Ed. 2, 439
ACTINIDIACEAE 猕猴桃科 mi hou tao ke Li Jianqiang (李建强)1, Li Xinwei (李新伟)1; Djaja Djendoel Soejarto2 Trees, shrubs, or woody vines. Leaves alternate, simple, shortly or long petiolate, not stipulate. Flowers bisexual or unisexual or plants polygamous or functionally dioecious, usually fascicled, cymose, or paniculate. Sepals (2 or 3 or)5, imbricate, rarely valvate. Petals (4 or)5, sometimes more, imbricate. Stamens 10 to numerous, distinct or adnate to base of petals, hypogynous; anthers 2- celled, versatile, dehiscing by apical pores or longitudinally. Ovary superior, disk absent, locules and carpels 3–5 or more; placentation axile; ovules anatropous with a single integument, 10 or more per locule; styles as many as carpels, distinct or connate (then only one style), generally persistent. Fruit a berry or leathery capsule. Seeds not arillate, with usually large embryos and abundant endosperm. Three genera and ca. 357 species: Asia and the Americas; three genera (one endemic) and 66 species (52 endemic) in China. Economically, kiwifruit (Actinidia chinensis var. deliciosa) is an important fruit, which originated in central China and is especially common along the Yangtze River (well known as yang-tao). Now, it is widely cultivated throughout the world. For additional information see the paper by X. W. Li, J. Q. Li, and D. D. Soejarto (Acta Phytotax. Sin. 45: 633–660. 2007). Liang Chou-fen, Chen Yong-chang & Wang Yu-sheng. 1984. Actinidiaceae (excluding Sladenia). In: Feng Kuo-mei, ed., Fl. Reipubl. Popularis Sin. 49(2): 195–301, 309–334. 1a. Trees or shrubs; flowers bisexual or plants functionally dioecious .................................................................................. 3. Saurauia 1b. -
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. -
UC Davis Dermatology Online Journal
UC Davis Dermatology Online Journal Title Penicillamine-associated cutis laxa and milia en plaque - case report and review of cutaneous changes associated with penicillamine Permalink https://escholarship.org/uc/item/47p4d8zv Journal Dermatology Online Journal, 22(5) Authors Vajdi, Tina Lee, Wiggin Wu Paravar, Taraneh Publication Date 2016 DOI 10.5070/D3225030951 License https://creativecommons.org/licenses/by-nc-nd/4.0/ 4.0 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Volume 22 Number 5 May 2016 Photo Vignette Penicillamine-associated cutis laxa and milia en plaque - case report and review of cutaneous changes associated with penicillamine Tina Vajdi1, Wiggin Wu Lee2, Taraneh Paravar2 Dermatology Online Journal 22 (5): 12 1University of California, San Diego School of Medicine 2Department of Dermatology, University of California, San Diego Correspondence: Taraneh Paravar, MD Assistant Clinical Professor Department of Dermatology University of California, San Diego 8899 University Center Lane, Suite 350 San Diego, California 92122, USA Tel. (858) 657-8322 E-mail: [email protected] Abstract Penicillamine-induced skin changes are rare and include: hypersensitivity reactions, autoimmune reactions, and cutaneous elastoses. We report a case of a 73-year-old man with cystinuria taking penicillamine for over 50 years who presented with penicillamine-induced cutis laxa and milia en plaque. A brief review of penicillamine induced skin changes, specifically cutis laxa and milia en plaque, is presented. Key Words: penicillamine, elastic tissue, cystinuria, cutis laxa, milia en plaque Introduction Penicillamine is a chelating agent commonly used to treat cystinuria and Wilson disease. Cystinuria is a genetic disorder in which patients lack the cysteine amino acid transporter. -
1St International Conference of Translational Medicine
American Journal of Translational Medicine Vol 3 Issue 1, 2019 ISSN 2474-7378 (P) & 2474-7386 (O) A J CONFERENCE T M Proceedings of st 2 1 International Conference of Translational Medicine 0 1 You-Ping Deng1, JIanxin Gao2, Zhiqian Hu3, Zhong Li4, Guo-Tong Xu5, Gang-Ming Zou6 9 1University of Hawaii School of Medicine, Honolulu, USA 2Shanghai Jiao Tong University, Shanghai, China. 3Shanghai Changzheng Hospital, Shanghai, China 4Shanghai Cell Therapy Institute, Shanghai, China 5Tongji University, Shanghai, China 6American Journal of Translational Medicine. Honolulu, USA *Correspondence: G.M.Z. (email: [email protected]) Conference Chair: Gang-Ming Zou, MD, PhD Conference Co-Chair: Guo-Tong Xu, PhD Conference General Secretary: You-Ping Deng, PhD Programs: Program A Translational Oncology (Abstract A001-A019) Program leader: Zhi-qian Hu, MD Program B Stem cell and Regenerative medicine (Abstract B001-B012) Program leader Guo-Tong Xu, PhD Program C Medicine and Health (Abstract C001-C013) Program leader: Gang-Ming Zou, MD, PhD Scientific Advisors: Zhong-Chao Han (China). Zhi-Qian Hu (China), Victor Lobanenkov, PhD (USA), Organizing Committee: You-Ping Deng, PhD (USA), Cunyi Fan (China), Jian-Xin Gao, PhD (China), Zhi-Quan Hu, MD (China), Wen-Yang Hu, MD (USA), Zhong LI (China), Daniel Scherman, PhD, (France), Georges Uzan, PhD (France), Liaonan Zou, MD, PhD (China), Guo-Tong Xu (China), Gang-Ming Zou (USA), 1 Hawaii Gangze InC., Publisher, Honolulu, USA American Journal of Translational Medicine Vol 3 Issue 1, 2019 ISSN 2474-7378 (P) & 2474-7386 (O) Conference Committee: You-Ping Deng, PhD, Guo-Tong Xu, MD, Gang-Ming Zou, MD, PhD Finance Chair and Treasurer: You-Ping Deng PhD, Finance and Treasure Committee: You-Ping Deng PhD, Gang-Ming Zou, MD, PhD. -
Anetoderma Secondary to Mid-Dermal Elastolysis
Anetoderma Secondary to Mid-dermal Elastolysis Gabriela A. Maloney, BS,* Jane James, MD, PhD,** Michael Welsch, MD,** Marylee Braniecki, MD** *Midwestern University, Downers Grove, IL **Pathology Department, University of Illinois Hospital & Health Sciences System, Chicago, IL Abstract Anetoderma usually presents as circumscribed, 1 cm to 2 cm patches and plaques of flaccid skin secondary to loss of dermal elastic tissue. Lesions often occur in the neck, upper extremities, chest, and back. On histopathology, one sees complete loss of dermal elastin involving the papillary and reticular dermis, with infiltration of plasma cells and histiocytes. A 40-year-old female with no significant medical history presented with multiple round, 1 cm to 2 cm lesions scattered on her upper back and chest. Skin biopsy demonstrated elastic-fiber loss localized to the mid-dermis along with a lymphohistiocytic infiltrate with elastophagocytosis and active inflammatory phase in the papillary and mid-reticular dermis. The histopathological findings were consistent with mid-dermal elastolysis with advancing inflammation, and the clinical features were consistent with anetoderma. The microscopic examination revealed an active inflammatory phase of mid-dermal elastolysis, supporting the postulated theory that MDE may be part of a continuous spectrum with anetoderma. Case Report by lax, wrinkled skin with underlying palpable biopsy and elastic-fiber staining demonstrated A 40-year-old female with no significant medical depression (Figure 1). They were often preceded elastic-fiber loss in the mid-dermis along with history presented with multiple round, 1 cm to by two to six months of local erythema and had a lymphohistiocytic infiltrate with evidence 2 cm lesions scattered throughout the upper increased in number over the past two years. -
Dermatose Degenerativa Induzida Por D-Penicilamina Em Paciente Com Doença De Wilson
Revista SPDV 76(2) 2018; D-Penicillamine induced degenerative dermopathy; Rui Pedro Santos, Joana Gomes, Celeste Brito. Caso Clínico Dermatose Degenerativa Induzida por D-penicilamina em Paciente com Doença de Wilson Rui Pedro Santos1, Joana Gomes2, Celeste Brito2 1Interno de Dermatovenereologia/Resident, Dermatovenereology, Hospital de Braga, Braga, Portugal 2Especialista de Dermatovenereologia/Specialist of Dermatovenereology, Hospital de Braga, Braga, Portugal RESUMO – As dermatoses degenerativas induzidas por D-penicilamina incluem, entre outras, a elastose perfurante serpiginosa e o pseudo-pseudoxantoma elástico. A elastose perfurante serpiginosa é uma doença perfurante rara caracterizada pela elimi- nação transepidérmica de fibras elásticas anormais. Esta condição pode ser idiopática, reativa ou induzida por D-penicilamina, habitualmente utilizada para o tratamento da doença de Wilson, cistinúria, artrite reumatóide ou esclerose sistémica. Manifesta- ções cutâneas semelhantes a pseudoxantoma elástico mas sem história familiar e mutações do gene ABCC6 foram identificadas como sendo uma dermatose induzida por D-penicilamina e designada de pseudo-pseudoxantoma elástico. Descreve-se o caso de uma mulher de 17 anos tratada por vários anos com D-penicilamina para doença de Wilson, com pápulas assintomáticas, algumas cor de pele e hiperqueratósicas e outras macias e amareladas, na região cervical e face. A histopatolo- gia mostrou a eliminação transepidérmica de fibras elásticas espessadas, em forma de dentes de serra. Estes achados sugeriram uma dermopatia induzida por D-penicilamina e os autores consideraram o diagnóstico de elastose perfurante serpiginosa e pseudo-pseudoxantoma elástico no mesmo paciente. O fármaco foi alterado para acetato de zinco sem lesões novas, mas com manutenção das lesões existentes no seguimento a 1 ano. -
Ex Vivo Analysis of DNA Repair Targeting in Extreme Rare Cutaneous Apocrine Sweat Gland Carcinoma
www.oncotarget.com Oncotarget, 2021, Vol. 12, (No. 11), pp: 1100-1109 Research Paper Ex vivo analysis of DNA repair targeting in extreme rare cutaneous apocrine sweat gland carcinoma Rami Mäkelä1, Ville Härmä1,2, Nibal Badra Fajardo3, Greg Wells2, Zoi Lygerou3, Olle Sangfelt4, Juha Kononen5 and Juha K. Rantala1,2 1Misvik Biology Oy, Turku, Finland 2University of Sheffield, Department of Oncology and Metabolism, Sheffield, UK 3University of Patras, Laboratory of General Biology, Patras, Greece 4Karolinska Institutet, Department of Cell and Molecular Biology, Stockholm, Sweden 5Docrates Cancer Hospital, Helsinki, Finland Correspondence to: Juha K. Rantala, email: [email protected] Keywords: cutaneous apocrine sweat gland carcinoma; ex vivo drug screening; DNA repair; PALB2; rare cancer Received: July 30, 2020 Accepted: May 03, 2021 Published: May 25, 2021 Copyright: © 2021 Mäkelä et al. This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. ABSTRACT Cutaneous apocrine carcinoma is an extreme rare malignancy derived from a sweat gland. Histologically sweat gland cancers resemble metastatic mammary apocrine carcinomas, but the genetic landscape remains poorly understood. Here, we report a rare metastatic case with a PALB2 aberration identified previously as a familial susceptibility gene for breast cancer in the Finnish population. As PALB2 exhibits functions in the BRCA1/2-RAD51-dependent homologous DNA recombination repair pathway, we sought to use ex vivo functional screening to explore sensitivity of the tumor cells to therapeutic targeting of DNA repair. Drug screening suggested sensitivity of the PALB2 deficient cells to BET-bromodomain inhibition, and modest sensitivity to DNA-PKi, ATRi, WEE1i and PARPi. -
Thermally Induced Actinidine Production in Biological Samples Qingxing Shi, Yurong He, Jian Chen,* and Lihua Lu*
pubs.acs.org/JAFC Article Thermally Induced Actinidine Production in Biological Samples Qingxing Shi, Yurong He, Jian Chen,* and Lihua Lu* Cite This: https://dx.doi.org/10.1021/acs.jafc.0c02540 Read Online ACCESS Metrics & More Article Recommendations *sı Supporting Information ABSTRACT: Actinidine, a methylcyclopentane monoterpenoid pyridine alkaloid, has been found in many iridoid-rich plants and insect species. In a recent research on a well-known actinidine- and iridoid-producing ant species, Tapinoma melanocephalum (Fabricius) (Hymenoptera: Formicidae), no actinidine was detected in its hexane extracts by gas chromatography−mass spectrometry analysis using a common sample injection method, but a significant amount of actinidine was detected when a solid injection technique with a thermal separation probe was used. This result led us to hypothesize that heat can induce the production of actinidine in iridoid-rich organisms. To test our hypothesis, the occurrence of actinidine was investigated in four iridoid-rich organisms under different sample preparation temperatures, including two ant species, T. melanocephalum and Iridomyrmex anceps Roger (Hymenoptera: Formicidae), and two plant species, Actinidia polygama Maxim (Ericales: Actinidiaceae) and Nepeta cataria L. (Lamiales: Lamiaceae). Within a temperature range of 50, 100, 150, 200, and 250 °C, no actinidine was detected at 50 °C, but it appeared at temperatures above 100 °C for all four species. A positive relationship was observed between the heating temperature and actinidine production. The results indicate that actinidine could be generated at high temperatures. We also found that the presence of methylcyclopentane monoterpenoid iridoids (iridodials and nepetalactone) was needed for thermally induced actinidine production in all tested samples.