Livedoid Vasculopathy: a Compelling Diagnosis
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Review Cutaneous Patterns Are Often the Only Clue to a a R T I C L E Complex Underlying Vascular Pathology
pp11 - 46 ABstract Review Cutaneous patterns are often the only clue to a A R T I C L E complex underlying vascular pathology. Reticulate pattern is probably one of the most important DERMATOLOGICAL dermatological signs of venous or arterial pathology involving the cutaneous microvasculature and its MANIFESTATIONS OF VENOUS presence may be the only sign of an important underlying pathology. Vascular malformations such DISEASE. PART II: Reticulate as cutis marmorata congenita telangiectasia, benign forms of livedo reticularis, and sinister conditions eruptions such as Sneddon’s syndrome can all present with a reticulate eruption. The literature dealing with this KUROSH PARSI MBBS, MSc (Med), FACP, FACD subject is confusing and full of inaccuracies. Terms Departments of Dermatology, St. Vincent’s Hospital & such as livedo reticularis, livedo racemosa, cutis Sydney Children’s Hospital, Sydney, Australia marmorata and retiform purpura have all been used to describe the same or entirely different conditions. To our knowledge, there are no published systematic reviews of reticulate eruptions in the medical Introduction literature. he reticulate pattern is probably one of the most This article is the second in a series of papers important dermatological signs that signifies the describing the dermatological manifestations of involvement of the underlying vascular networks venous disease. Given the wide scope of phlebology T and its overlap with many other specialties, this review and the cutaneous vasculature. It is seen in benign forms was divided into multiple instalments. We dedicated of livedo reticularis and in more sinister conditions such this instalment to demystifying the reticulate as Sneddon’s syndrome. There is considerable confusion pattern. -
Update on Vasculitis: Overview and Relevant
An Bras Dermatol. 2020;95(4):493---507 Anais Brasileiros de Dermatologia www.anaisdedermatologia.org.br REVIEW Update on vasculitis: overview and relevant dermatological aspects for the clinical and ଝ,ଝଝ histopathological diagnosis --- Part II a,∗ b Thâmara Cristiane Alves Batista Morita , Paulo Ricardo Criado , b a a Roberta Fachini Jardim Criado , Gabriela Franco S. Trés , Mirian Nacagami Sotto a Department of Dermatology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil b Dermatology Discipline, Faculdade de Medicina do ABC, Santo André, SP, Brazil Received 8 December 2019; accepted 28 April 2020 Available online 24 May 2020 Abstract Vasculitis is a group of several clinical conditions in which the main histopathological KEYWORDS finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main derma- Anti-neutrophil tological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cytoplasmic cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also antibodies; discussed. Churg-Strauss © 2020 Sociedade Brasileira de Dermatologia. Published by Elsevier Espana,˜ S.L.U. This is an syndrome; open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). Henoch-Schönlein purple; Leukocytoclastic cutaneous vasculitis; Systemic vasculitis; Vasculitis; Vasculitis associated with lupus of the central nervous system ଝ How to cite this article: Morita TCAB, Criado PR, Criado RFJ, Trés GFS, Sotto MN. Update on vasculitis: overview and relevant dermato- logical aspects for the clinical and histopathological diagnosis --- Part II. An Bras Dermatol. 2020;95:493---507. ଝଝ Study conducted at the Department of Dermatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. -
Update on Vasculitis: Overview and Relevant
An Bras Dermatol. 2020;95(4):493---507 Anais Brasileiros de Dermatologia www.anaisdedermatologia.org.br REVIEW Update on vasculitis: overview and relevant dermatological aspects for the clinical and ଝ,ଝଝ histopathological diagnosis --- Part II a,∗ b Thâmara Cristiane Alves Batista Morita , Paulo Ricardo Criado , b a a Roberta Fachini Jardim Criado , Gabriela Franco S. Trés , Mirian Nacagami Sotto a Department of Dermatology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil b Dermatology Discipline, Faculdade de Medicina do ABC, Santo André, SP, Brazil Received 8 December 2019; accepted 28 April 2020 Available online 24 May 2020 Abstract Vasculitis is a group of several clinical conditions in which the main histopathological KEYWORDS finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main derma- Anti-neutrophil tological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cytoplasmic cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also antibodies; discussed. Churg-Strauss © 2020 Sociedade Brasileira de Dermatologia. Published by Elsevier Espana,˜ S.L.U. This is an syndrome; open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). Henoch-Schönlein purple; Leukocytoclastic cutaneous vasculitis; Systemic vasculitis; Vasculitis; Vasculitis associated with lupus of the central nervous system ଝ How to cite this article: Morita TCAB, Criado PR, Criado RFJ, Trés GFS, Sotto MN. Update on vasculitis: overview and relevant dermato- logical aspects for the clinical and histopathological diagnosis --- Part II. An Bras Dermatol. 2020;95:493---507. ଝଝ Study conducted at the Department of Dermatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. -
The Management of Patients with Systemic Lupus Erythematosus (SLE)
Systemic lupus erythematosus (SLE) Rudolf Horváth, M.D., Ph.D. Andrea Čaničová, M.D. Dpt. of pediatric and adult rheumatology Faculty Hospital Motol Prague Introduction • SLE is a chronic inflammatory disease most commonly affecting young women. • SLE is characterized by hyperreactivity of B cells and overproduction of organ nonspecific autoantibodies. • The cause or causes of SLE remains unknown, with several environmental factors as important potential triggers for those with underlying genetic influences. • Typically the course of the disease is a series of remissions and exacerbations. • With good management, the ten years survival may be over 90%. Clinical picture • Variable • Systemic features - fever, loss of weight, fatigue (50-100% ) • Organ involvement – different extension and severity Skin involement • LE-specific skin lesions • LE-non-specific lesions – Acute cutaneous LE - malar – Cutaneous vascular disease – “butterfly” rash, generalized vasculitis (leukocytoclastic, palpable erythema purpura), urticarial vasculitis, – Subacute – annular, periarteritis nodosa–like papulosquamous (psoriasiform) – Vasculopathy - atrophy blanche, – Chronic – „classic” discoid LE , periungual telangiectasia, livedo localized discoid, generalized reticularis, thrombophlebitis, discoid, hypertrophic (verrucous) Raynaud phenomenon, discoid LE, lupus profundus, mucosal erythromelalgia LE – Alopecia (non-scarring) – “lupus hair”, alopecia areata – LE-non-specific bullous lesions- epidermolysis bullosa–like bullous LE, dermatitis herpetiformis–like -
Dermatological Indications of Disease - Part II This Patient on Dialysis Is Showing: A
“Cutaneous Manifestations of Disease” ACOI - Las Vegas FR Darrow, DO, MACOI Burrell College of Osteopathic Medicine This 56 year old man has a history of headaches, jaw claudication and recent onset of blindness in his left eye. Sed rate is 110. He has: A. Ergot poisoning. B. Cholesterol emboli. C. Temporal arteritis. D. Scleroderma. E. Mucormycosis. Varicella associated. GCA complex = Cranial arteritis; Aortic arch syndrome; Fever/wasting syndrome (FUO); Polymyalgia rheumatica. This patient missed his vaccine due at age: A. 45 B. 50 C. 55 D. 60 E. 65 He must see a (an): A. neurologist. B. opthalmologist. C. cardiologist. D. gastroenterologist. E. surgeon. Medscape This 60 y/o male patient would most likely have which of the following as a pathogen? A. Pseudomonas B. Group B streptococcus* C. Listeria D. Pneumococcus E. Staphylococcus epidermidis This skin condition, erysipelas, may rarely lead to septicemia, thrombophlebitis, septic arthritis, osteomyelitis, and endocarditis. Involves the lymphatics with scarring and chronic lymphedema. *more likely pyogenes/beta hemolytic Streptococcus This patient is susceptible to: A. psoriasis. B. rheumatic fever. C. vasculitis. D. Celiac disease E. membranoproliferative glomerulonephritis. Also susceptible to PSGN and scarlet fever and reactive arthritis. Culture if MRSA suspected. This patient has antithyroid antibodies. This is: • A. alopecia areata. • B. psoriasis. • C. tinea. • D. lichen planus. • E. syphilis. Search for Hashimoto’s or Addison’s or other B8, Q2, Q3, DRB1, DR3, DR4, DR8 diseases. This patient who works in the electronics industry presents with paresthesias, abdominal pain, fingernail changes, and the below findings. He may well have poisoning from : A. lead. B. -
Mallory Prelims 27/1/05 1:16 Pm Page I
Mallory Prelims 27/1/05 1:16 pm Page i Illustrated Manual of Pediatric Dermatology Mallory Prelims 27/1/05 1:16 pm Page ii Mallory Prelims 27/1/05 1:16 pm Page iii Illustrated Manual of Pediatric Dermatology Diagnosis and Management Susan Bayliss Mallory MD Professor of Internal Medicine/Division of Dermatology and Department of Pediatrics Washington University School of Medicine Director, Pediatric Dermatology St. Louis Children’s Hospital St. Louis, Missouri, USA Alanna Bree MD St. Louis University Director, Pediatric Dermatology Cardinal Glennon Children’s Hospital St. Louis, Missouri, USA Peggy Chern MD Department of Internal Medicine/Division of Dermatology and Department of Pediatrics Washington University School of Medicine St. Louis, Missouri, USA Mallory Prelims 27/1/05 1:16 pm Page iv © 2005 Taylor & Francis, an imprint of the Taylor & Francis Group First published in the United Kingdom in 2005 by Taylor & Francis, an imprint of the Taylor & Francis Group, 2 Park Square, Milton Park Abingdon, Oxon OX14 4RN, UK Tel: +44 (0) 20 7017 6000 Fax: +44 (0) 20 7017 6699 Website: www.tandf.co.uk All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior permission of the publisher or in accordance with the provisions of the Copyright, Designs and Patents Act 1988 or under the terms of any licence permitting limited copying issued by the Copyright Licensing Agency, 90 Tottenham Court Road, London W1P 0LP. Although every effort has been made to ensure that all owners of copyright material have been acknowledged in this publication, we would be glad to acknowledge in subsequent reprints or editions any omissions brought to our attention. -
Cutaneous Vasculitis
Cutaneous Vasculitis Authors: Lorinda Chung, M.D. and David Fiorentino1, M.D., Ph.D. Creation date: March 2005 Scientific editor: Prof Loïc Guillevin 1Department of Dermatology, Division of Rheumatology and Immunology, Stanford University School of Medicine, 900 Blake Wilbur Drive W0074, Stanford, CA. USA. [email protected] Abstract Keywords Definition Classification / Etiology Approach to the Patient Treatment Future Directions References Abstract Cutaneous vasculitis is a histopathologic entity characterized by neutrophilic transmural inflammation of the blood vessel wall associated with fibrinoid necrosis, termed leukocytoclastic vasculitis (LCV). Clinical manifestations of cutaneous vasculitis occur when small and/or medium vessels are involved. Small vessel vasculitis can present as palpable purpura, urticaria, pustules, vesicles, petechiae, or erythema multiforme-like lesions. Signs of medium vessel vasculitis include livedo reticularis, ulcers, subcutaneous nodules, and digital necrosis. The frequency of vasculitis with skin involvement is unknown. Vasculitis can involve any organ system in the body, ranging from skin-limited to systemic disease. Although vasculitis is idiopathic in 50% of cases, common associations include infections, inflammatory diseases, drugs, and malignancy. The management of cutaneous vasculitis is based on four sequential steps: confirming the diagnosis with a skin biopsy, evaluating for systemic disease, determining the cause or association, and treating based on the severity of disease. Keywords Cutaneous vasculitis, leukocytoclastic vasculitis Definition Vasculitis is inflammation of the blood vessel Classification / Etiology wall that leads to various clinical manifestations The classification schemes for the vasculitides depending on which organ systems are involved. are based on several criteria, including the size Cutaneous vasculitis is a histopathologic entity of the vessel involved, clinical and characterized by neutrophilic transmural histopathologic features, and etiology. -
Rush University Medical Center, May 2004
CHICAGO DERMATOLOGICAL SOCIETY RUSH UNIVERSITY MEDICAL CENTER CHICAGO, ILLINOIS MAY 19, 2004 TABLE OF CONTENTS Case # Title Page 1. Urticarial Vasculitis 1 2. Subcorneal Pustular Dermatosis 3 3. Animal-Type Melanoma 6 4. Multiple Clustered Dermatofibromas 8 5. Erythrokeratoderma Variabilis 10 6. “Unknown” 13 7. Anhidrotic Ectodermal Dysplasia 14 8. Linear Lichen Planus 17 9. Atypical Sweet’s Syndrome 19 10. Pancreatic Panniculitis 22 11. Bullous Sweet’s Syndrome 24 12. Plane Xanthoma 28 13. Hereditary Hemorrhagic Telangiectasia 31 14. Generalized Schamberg’s Disease 34 15. Eruptive Cherry Angiomas 37 16. Henoch-Schönlein Purpura 40 17. Generalized Granuloma Annulare 43 18. Thyroid Dermopathy 45 19. Lichen Spinulosus 47 20. Cystic Hygroma 49 Page 1 of 51 Case # 1 CHICAGO DERMATOLOGICAL SOCIETY RUSH UNIVERSITY MEDICAL CENTER CHICAGO, ILLINOIS MAY 19, 2004 CASE PRESENTED BY: Michael D. Tharp, M.D. and Alix J. Charles, M.D. History: This 51 year old white male was referred to our department for evaluation and treatment of a burning and pruritic erythematous eruption of seven months duration. The patient reported that lesions would appear on the trunk and extremities and last for more than twenty-four hours before resolving with hyperpigmentation. The eruption was also associated with swelling and joint pain of the hands. Treatment with zyrtec, allegra, clarinex, atarax, and medrol dose packs had been met with little success. Past Medical History: Family history of systemic lupus (daughter). Otherwise unremarkable. Physical Findings: The extremities, chest, and back display dozens of discrete and confluent erythematous and purpuric, non-blancheable macules and plaques. Some lesions have an annular configuration. -
Atrophie Blanche: Is It Associated with Venous Disease Or Livedoid Vasculopathy?
Zurich Open Repository and Archive University of Zurich Main Library Strickhofstrasse 39 CH-8057 Zurich www.zora.uzh.ch Year: 2014 Atrophie blanche: Is it associated with venous disease or livedoid vasculopathy? Alavi, Afsaneh ; Hafner, Jurg ; Dutz, Jan P ; Mayer, Dieter ; Sibbald, R Gary ; Criado, Paulo Ricardo ; Senet, Patricia ; Callen, Jeffery P ; Phillips, Tania J ; Romanelli, Marco ; Kirsner, RobertS Abstract: PURPOSE: The purpose of this learning activity is to provide information about the etiology and treatment of atrophie blanche. TARGET AUDIENCE: This continuing education activity is intended for physicians and nurses with an interest in skin and wound care. OBJECTIVES: After participating in this educational activity, the participant should be better able to:1. Discuss the pathophysiology of atrophie blanche.2. Explore treatment options for livedoid vasculopathy. ABSTRACT: Atrophie blanche (AB) is a porcelain-white scar that may be seen at the base of a healed ulcer or in association with livedoid vasculopathy (LV). The term AB originally had been used synonymously with LV, whereas LV is a noninflammatory thrombotic condition presenting as either a primary or secondary event (often associated with coagulation). DOI: https://doi.org/10.1097/01.ASW.0000455098.98684.95 Posted at the Zurich Open Repository and Archive, University of Zurich ZORA URL: https://doi.org/10.5167/uzh-106873 Journal Article Published Version Originally published at: Alavi, Afsaneh; Hafner, Jurg; Dutz, Jan P; Mayer, Dieter; Sibbald, R Gary; Criado, Paulo Ricardo; Senet, Patricia; Callen, Jeffery P; Phillips, Tania J; Romanelli, Marco; Kirsner, Robert S (2014). Atrophie blanche: Is it associated with venous disease or livedoid vasculopathy? Advances in Skin Wound Care, 27(11):518-524. -
** No Patient Handout Vasculitis
** no patient handout Vasculitis Synopsis Vasculitis is a nonspecific term that encompasses a large and heterogeneous group of disorders that are characterized by inflammation of blood vessels. No uniform classification system for vasculitis exists, although subtypes are often categorized by the size of the vessels involved, the type of circulating immune complexes, and other histopathologic and clinical features. In many cases, damage to the blood vessels results in leakage of red blood cells and the development of palpable purpura on the skin. Other common cutaneous signs of vasculitis include skin ulcerations, Raynaud phenomenon, and livedo reticularis. It is important to distinguish a primary systemic vasculitis from one associated with medications, infection, malignancy, or a connective tissue disorder, as the best course of treatment may differ. Drug-induced vasculitis should be quickly recognized and managed by removing the causative medication. Ongoing drug-induced vasculitis can lead to glomerulonephritis, pulmonary hemorrhage, mononeuritis multiplex, and other complications of end-organ involvement. Several types of vasculitis are more likely than others to be mistaken for cellulitis. Among these are polyarteritis nodosa and variants of leukocytoclastic (hypersensitivity) vasculitis such as erythema elevatum diutinum and urticarial vasculitis. Kawasaki disease and Henoch- Schönlein purpura may present with some lesions that mimic cellulitis, but these diseases occur predominantly in children. On rarer occasions, other vasculidities such as granulomatosis with polyangiitis, Behçet syndrome, cryoglobulinemia, and livedoid vasculopathy may present similarly to cellulitis. Other primary vasculitic syndromes include eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, Takayasu arteritis, and giant cell (temporal) arteritis. Vasculitis may occur in association with such diseases as rheumatoid arthritis, systemic lupus erythematosus, and dermatomyositis. -
CUTANEOUS VASCULITIS Katharine Warburton ST6 Dermatology AIMS
CUTANEOUS VASCULITIS Katharine Warburton ST6 Dermatology AIMS Clinical cases introduction ‘The theory’ Categorising cutaneous vasculitis Features presenting in the skin Mimics/pitfalls How to initially manage and investigate in GIM When to involve dermatology When is biopsy required Management principles Back to cases CASES CASE 1 57 year old female Mild chronic ITP, untreated (plts 80) Obesity Had 3/7 trimethoprim from GP for uncomplicated UTI Completed 12 days ago Presents with new rash after travelling up from Cornwall Well Obs normal Urine dip normal Initial bloods ok CASE 2 22 year old student Rapidly extending rash 1 wk GP gave antibiotics for folliculitis but progressed Feels ‘not right’, bit achey. Crampy abdo pain. BP 132/75 Urine dip trace of protein Initial bloods ok, vasculitis screen pending CASE 3 Female, 54 Acute presentation ascites, leg swelling, vomiting Hep C positivity identified Perforated gastric ulcer, multi organ failure ‘THE THEORY’ Vasculitis = inflammation of blood vessel CLASSIFYING VASCULITIS Small vessels <50micrometers Medium vessels 50-150 micrometer Large vessel CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary IgA vasculitis (Henoch-Schonlein Purpura, HSP) Urticarial vasculitis Cryoglobulinaemic ANCA associated Primary systemic vasculitidies (<4%) Polyarteritis nodosa Nodular CATEGORISING CUTANEOUS VASCULITIS Reactive/secondary (cutaneous small vessel vasculitis) • IgAMostly vasculitis limited (Henoch to skin-Schonlein Purpura, HSP) • 7-10 days after exposure to -
Small Vessel Vasculitis Revista Colombiana De Reumatología Vol
VOL. 14 No. 3 - 2007 SMALL VESSEL VASCULITIS REVISTA COLOMBIANA DE REUMATOLOGÍA VOL. 14 No. 3, Septiembre 2007, pp. 187-205 © 2007, Asociación Colombiana de Reumatología ARTÍCULO DE REVISIÓN Small vessel vasculitis History, classification, etiology, histopathology, clinic, diagnosis and treatment Antonio Iglesias Gamarra1, Eric L. Matteson2, José Félix Restrepo3 Summary Resumen Small-vessel vasculitis is a convenient descriptor El término vasculitis de pequeños vasos descri- for a wide range of diseases characterized by vascular be a un grupo de enfermedades caracterizadas por inflammation of the venules, capillaries, and/or inflamación de vénulas, capilares y/o arteriolas con arterioles with pleomorphic clinical manifestations. manifestaciones clínicas pleomórficas. El fenotipo clí- The classical clinical phenotype is leukocytoclastic vas- nico clásico es la vasculitis leucocitoclástica con púr- culitis with palpable purpura, but manifestations vary pura palpable, pero con manifestaciones que varían widely depending upon the organs involved. His- ampliamente dependiendo del órgano comprometido. topathologic examination in leukocytoclastic vasculi- La histología en la vasculitis leucocitoclástica revela tis reveals angiocentric segmental inflammation, una inflamación segmentaria angiocéntrica, necrosis fibrinoid necrosis, and a neutrophilic infiltrate around fibrinoide e infiltrado neutrofílico alrededor de los the blood vessel walls with erythrocyte extravasation. vasos sanguíneos, con extravasación de eritrocitos. The etiology of small-vessel vasculitis is unknown in La etiología de las vasculitis de pequeños vasos es many cases, but in others, drugs, post viral syndromes, desconocida, en muchos casos, pero en otros se ha malignancy, primary vasculitis such as microscopic pol- asociado con drogas, síndromes post virales, neo- yarteritis, and connective tissue disorders are associ- plasias, vasculitis primarias como la poliarteritis mi- ated.