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Ward Rounds in DERMATOLOGY

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Ward Rounds in DERMATOLOGY

The Health Sciences PublisherNew Delhi | London | Panama

Bela J Shah MD

Professor and HeadDepartment of Dermatology,

Sexually Transmitted Infection (STI) and LeprosyBJ Medical College and Civil Hospital

Ahmedabad, Gujarat, India

Santosh Rathod MD (Skin and VD) DNB

Associate Professor Department of Dermatology

Smt Nathiba Hargovandas Lakhmichand (NHL) Municipal Medical College and VS Hospital

Ahmedabad, Gujarat, India

Foreword Sudhir Pujara

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Jaypee Brothers Medical Publishers (P) Ltd.

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Ward Rounds in Dermatology

First Edition: 2017

ISBN: 978-93-86322-68-5

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© 2017, Jaypee Brothers Medical Publishers

The views and opinions expressed in this book are solely those of the original contributor(s)/author(s) and do not necessarily represent those of editor(s) of the book.All rights reserved. No part of this publication may be reproduced, stored or transmitted in any form or by any means, electronic, mechanical, photo copying, recording or otherwise, without the prior permission in writing of the publishers. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book.Medical knowledge and practice change constantly. This book is designed to provide accurate, authoritative information about the subject matter in question. However, readers are advised to check the most current information available on procedures included and check information from the manufacturer of each product to be administered, to verify the recommended dose, formula, method and duration of administration, adverse effects and contra indications. It is the responsibility of the practitioner to take all appropriate safety precautions. Neither the publisher nor the author(s)/editor(s) assume any liability for any injury and/or damage to persons or property arising from or related to use of material in this book.This book is sold on the understanding that the publisher is not engaged in providing professional medical services. If such advice or services are required, the services of a competent medical professional should be sought.Every effort has been made where necessary to contact holders of copyright to obtain permission to reproduce copyright material. If any have been inadvertently overlooked, the publisher will be pleased to make the necessary arrangements at the first opportunity.

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Dedicated toMy father, Late Sree Jaswantlal Shah;

my mother, Vanlila J Shah; my husband, Ushir Mashruwala; and

my son, Soham Mashruwala—Bela J Shah

Three lovely ladies of my life:My mother, Manjula;

my wife, Archana; and my daughter, Kanvi

—Santosh Rathod

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Foreword

Ward Rounds in Dermatology by Bela J Shah and Santosh Rathod, provides a refreshing view of several clinical presentations that generally require admission, and discusses all the diagnostic aspects and management in great details. Dedicated chapters on immunosuppressives, corticosteroids, antibiotics and signs in dermatology make reading interesting. Discussion on recent aspects (e.g. in relation to autoimmune-blistering disorders) improves the already rich value of the book. The book contains practically everything one would want to know about common, and some not-so-common clinical situations. It elaborates the theoretical discussions (which one would have anyway read elsewhere to build up the conceptual framework) and provides enough practical details. This work by two accomplished dermatologists would serve as a quick-reference source to recapitulate what one has already learned earlier; but it is not just a compilation of facts, it is much more. Dr Santosh Rathod has been known to me as a well-read doctor. I had a pleasure of being Dr Bela’s postgraduate examiner for MD degree. She had already demonstrated her potential then. I have also worked with her on some projects. I have always appreciated her dedication while working with her.

Sudhir Pujara MD DVD DDV (Bom)

Formerly, Professor and HeadDepartment of Dermatology

Smt Nathiba Hargovandas Lakhmichand (NHL) Municipal Medical CollegeAhmedabad, Gujarat, India

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Preface

Dermatology is a branch of medicine which caters mainly to outdoor patients. Compared to outdoor patients, dermatologists have less workload as far as indoor patients are concerned. The management of indoor patients takes a backseat many a times. Patients with skin failure need to be monitored round the clock. We have been using dexamethasone cyclophosphamide pulse (DCP) therapy and biologics. This requires admission of the patients in the ward. Every morning for every admitted patient, we have to have our own checklist which will help us to manage the indoor patients effectively. We have included all the aspects of history-taking in dermatology in the book. This book is a culmination of knowledge gathered from standard textbooks/journals and authors’ own experience. The information has been produced in the form of questions and answers to guide the residents in their postgraduate examinations. The chapter on the drugs is a concise synopsis for a quick revision. At the end, a chapter on frequently asked questions (FAQs) in examinations is included in the complete question-answer format. Ward Rounds in Dermatology is essentially designed and aimed to help the beginners and the practitioners alike. We hope that you all will appreciate our hard work, and the book makes reading enjoyable.

Bela J ShahSantosh Rathod

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Acknowledgments

The book is an amalgamation of the experience gained in clinical practice for over the last 28 years (Dr Bela J Shah), and of an extensive study. We are grateful to a number of people, who have helped us during preparation of the script, without whom this journey would have been impossible. First and foremost, I (Bela J Shah) thank my revered teacher Dr Bharat H Shah, who has been a great guide, philosopher and teacher par excellence. With his blessings only I am able to pen down this book. We would like to acknowledge the efforts of our postgraduates who were involved at every stage. We thank our residents, Drs Uzzaif Mansuri, Ankan Gupta, Sonal Patel, Suyog Dhamale, Darshan Karia and Sonal Tibrewal for their untiring efforts. We would also like to acknowledge the efforts made by the residents of LG Hospital, Ahmedabad, Gujarat, India, especially Dr Shikha Shivhare. We are grateful to M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India, for their continued support in publishing this book. Lastly, we are thankful to the Almighty who helped us at every step.

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Contents

1. Autoimmune Vesiculobullous Disorders 1 Case A: Pemphigus Vulgaris 1 Origin, duration and Progress 1 Treatment History 2 Local Examination 2 History Taking and Clinical Examination 2 Clinical Examination 3 Laboratory Investigations 4 Case B: Bullous Pemphigoid 10 Management of a Case of Bullous Pemphigoid 10 Case C: Linear IgA Bullous Dermatosis 12 Case D: Epidermolysis Bullosa Acquisita 12 Our Experience 17

2. Drug Reactions 19 Case History 19 Physical Examination 20 Methotrexate Toxicity 27 Approach 28 Treatment of Toxicity 28

3. Systemic Lupus Erythematosus 30 Clinical History 30 Origin, duration and Progress 30 Negative History 31 Questioning for Mucocutaneous Manifestations 33 Musculoskeletal Manifestations 33 Physical Examination 34 Laboratory Evaluation of Patient with Systemic Lupus Erythematosus 36 Hematological Abnormalities 36 Urine Analysis 37 Renal Function Tests 38

4. Systemic Sclerosis, Dermatomyositis and other Connective Tissue Diseases 51

Chief Complaints 51 History of Present Illness 51 Physical Examination 52

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xiv Ward Rounds in Dermatology

Musculoskeletal System 53 Cutaneous Examination 54 Management of a Case of Systemic Sclerosis 66 Mixed Connective Tissue Disorder 71

5. Erythroderma 75 Examination 76 Sparing Signs in Erythroderma 76 Erythroderma in Neonates, Infants and Children 77 Lymphoma Work-up 81

6. Lepra Reactions 86 Clinical History 1 86 Clinical History 2 87 Terminologies 101

7. Cutaneous T-cell Lymphomas 106 Approach to a Case 106 History 106 Physical Examination 107 Skin Biopsy 107 Blood Test 108 Radiological Test 108 Lymph Node Biopsy 108 Differential Diagnosis 108 Treatment 109 Systemic Therapies 109 Systemic Chemotherapy 109 Allogeneic Hematopoietic Stem Cell Transplantation 109 Clinical Variants of Mycosis Fungoides 111

8. Immunosuppressants in Dermatology 113 Azathioprine 113 Cyclophosphamide 127 Pharmacology 127 Mechanism of Action 127 FDA-approved Indications in Dermatology 127 Off-label Dermatologic Uses 128 Contraindications 128 Adverse Effects 129 Drug Interactions 129 Therapeutic Guidelines 130 Cyclosporine 130 Pharmacology 130

9. Glucocorticosteroids 137 Mode of Action 137 Various Effects of Corticosteroids 137 Dermatologic Indications of Systemic Corticosteroids 138 Contraindications 139

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xvContents

Relative Potencies of Commonly used Corticosteroids 140 Mechanisms Responsible for Various Adverse Effects of Corticosteroids 140 Topical Corticosteroids 141 Contraindications of Topical Corticosteroids 143 FTU Guidelines for Adults 143 Adverse Effects of Topical Corticosteroids 143 Risk Factors for Systemic Effects and Local Atrophy 144

10. Antihistamines 146 H1 Antihistamines 146 H2 Antihistamines 149 Mast-cell Stabilizers 149

11. Use of Antibiotics in a Patient Admitted in Dermatology Ward 150 Culture and Sensitivity Pattern 150 Treatment 151 Vancomycin-resistant Staphylococcus aureus 153 Nosocomial Infections in Dermatology 153

12. Signs in Dermatology 156 Psoriasis 156 Connective Tissue Diseases 156 Autoimmune Bullous Disorders 157 Erythroderma 158

13. Frequently Asked Questions in Dermatology 159

14. Miscellaneous 168 Pyoderma Gangrenosum 168 Chief Complaints 168 History of Present Illness 168 Physical Examination 168 Antiphospholipid Antibody Syndrome 172 Physical Examination 172 Cutaneous Examination 172 Polyarteritis Nodosa 175 Chief Complaints 175 History of Present Illness 176 General Physical Examination 176 Cutaneous Examination 176 Laboratory Investigations 176 Granulomatosis with Polyangiitis (GPA)/Wegener’s Granulomatosis 177 Pulmonary 179 Subcutaneous Zygomycosis 182 Chief Complaints 182 History of Present Illness 182 Physical Examination 182

Index 187

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Chapter

Miscellaneous

14

PYODERMA GANGRENOSUM

CHIEF COMPLAINTS • Historyofrecurrentulcerationssincepast8years• Episodesofbloodinstooloffandonsincepast4–5years• Aggravationofcomplaintswithnewlesionssincepast15days.

HISTORY OF PRESENT ILLNESS A21-year-oldmalepresentedwithhistoryofrecurrentpainfulerythematousnodulesonhisrightfootwhichulceratedandrapidlyenlargedassociatedwithpusdischarge8yearsback.Fewmorenodulesappearedonleftlegwhichalsoulceratedandhealedwithscarring4–5yearsback.Atpresent,thepatientcamewithcomplaintoflesionswithsimilarmorphologysincepast15daysdevelopedoverleftbuttock,pubicregion,leftforearmandface. Patienthadhistoryofloosemotionswithbloodandmucusoffandonsincepast4–5years. Historyoffever,mildgradeoffandonnotassociatedwithchillsandrigorsoreveningriseandrelievedbymedications.Nohistoryof:• Jointpain• Weightloss• Abdominalpain• Photosensitivity• Pedaledema• Drynessofmouthoreyes.

Treatment History Thepatientwasadmittedpreviouslyforthesimilarcomplaint4yearsbackwhenhewasgivensteroidsandcapsulecyclosporine100mgtwicedaily.Hewasthenmaintainedontabletdapsone100mgatnightfor3yearsandwassymptomfree.Hestoppedtreatmentthereafterandafter1yearherelapsedwithnewulcersandwasadmittedforthesameonceagain. Therewasnoresponsetotreatmentwithvariousantibioticsandlowdosesofsteroidstakenfromoutsidebeforegettingadmitted. Nohistoryofsmokingoralcoholaddiction.Nohistoryofdiabetesmellitus(DM),hypertension(HTN),tuberculosis(TB)oranyothermajorsurgicalormedicalillness.Familyhistorywasnotsignificant.

PHYSICAL EXAMINATION Cutaneousexaminationshowedmultipleulcers,varyinginsizefrom4to15cmandpresentonleftcheek,leftforearmandleftbuttocksandmonspubisregion(Figs14.1to14.3).Borderswerewelldefined,elevated,

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169 Miscellaneous

Fig. 14.3 Single pustular lesion with ulceration in the center and surrounding erythema. Cribriform scarring from ulceration in the past

Fig. 14.2 Necrotic ulcer with surrounding erythema over left thigh

Fig. 14.1 Crusted lesion with scarring in the edges in pyoderma gangrenosum

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170 Ward Rounds in Dermatology

violaceousandedgeswereundermined.Floorwascoveredwithsloughandbloodydischarge.Singlefixed,tenderswellingoverlyingtherightparotidregionwasalsonoted. Systemicexaminationwasnormal. InvestigationsrevealedHbof11g%,totalleukocytecount(TLC)-24000/cu.mL,differentialleukocytecount-normalrange(DLC-N)69%,L31%anderythrocytesedimentationrate(ESR)72mminfirsthour.Fastingbloodsugarwas76mg%.Totalserumproteinandserumalbuminwere5.6g%and2.0grespectively.Serum bilirubinwas 1.0mg%, serum glutamate oxaloacetic transaminase (SGOT)-34 IU/mL, serumglutamatepyruvatetransaminase(SGPT)-45IU/mL.Screenforhumanimmunodeficiencyvirus(HIV)andhepatitiswasnegative. Mantouxandpathergytestswerenegative. Rheumatoidfactorwasalsonegative. ChestX-raywasnormal.Pusandstoolcultureweresterile. Skinbiopsyfromtheedgeoftheulcershowedlymphocyticvasculitis,extravasationofRBCsandabscessformationinthedermisconsistentwithpyodermaganagrenosum(PG).Colonoscopyrevealedredcolonicmucosawithmultiplediffuseulcerationsanderosions.Thereweremultiplepseudopolypsoverupperrectumandlowersigmoidcolon. Colonicbiopsywasnotdone.

Q.1. How will you investigate a case of pyoderma gangrenosum? Ans. Pyodermagangrenosumisadiagnosisofexclusionbecausenospecificcriteriahavebeendeterminedtoconfirmthediagnosis.Allotherpotentialcausesofsimilarlesionsmustbeexcludedpriortomakingthediagnosis.

Q.2. How will you manage a case of pyoderma gangrenosum? Ans. Patientwasstartedontreatmentwithdexamethasone1.5ccIVonceinthemorning,ranitidine150mgbidanddapsone100mgalongwithantibiotics.Localcareofulcersincludedpotassiumpermanganatesoaksanddressingswithtopicalantimicrobialagents. Theulcersstartedhealingwithin4weeksofstartingtreatment.Thedoseofsteroidwasgraduallytaperedandshiftedtooralprednisoloneandallotherdrugswerecontinuedinthesamedosage.All theulcerscompletelyhealed3monthsafterstartingthetreatment(Figs14.4and14.5). ThetreatmentofPGismostlyunsatisfactory.Theaimistotreattheunderlyingdisease. Numerousmodesoftreatmenthavebeentriedwithvaryingoutcomes.

Morefrequentlyusedtreatmentsinclude:• Topical,intralesionaloral,andpulsesteroidtherapy• Cyclophosphamide• Azathioprine• Antibiotics• Sulphadrugs• Dapsone• Clofazimine• Minocycline

Fig. 14.4 Two weeks post-treatment

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171 Miscellaneous

• Cyclosporine• Andintensivelocaltreatment• CyclosporinehasbeenreportedasaveryeffectivedrugforPGinthedoseof6–10mg/kg/daywithhealing

in1–3months.

Q.3. Which systemic conditions are associated with pyoderma gangrenosum frequently? Ans. Associatedsystemicdiseasesreportedare:• Ulcerativecolitis• Crohn’sdisease• Rheumatoidarthritis• Seronegativearthritis• Hematologicmalignancies• Collagenvasculardiseases• Monoclonalgammopathy• Hepaticandpancreaticdiseases• Wegener’sgranulomatosisand• Otherneutrophilicdermatosis.

Q.4. What is the prevalence of PG with ulcerative colitis? Ans. TheprevalenceofPGassociatedwithulcerativecolitishasbeenreportedtorangefrom30to60%.Symptomsofulcerativecolitismayprecede,followorbeconcomitantwithPG.

Figs 14.5A and B (A) Post-treatment; (B) Healed scar in case of pyoderma gangrenosum

A

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Q.5. Which differential diagnosis to be considered in a case suspected with pyoderma gangrenosum? Ans.• Antiphospholipidantibodysyndrome• Anthrax• Arterialinsufficiency• Acutefebrileneutrophilicdermatosis(Sweetsyndrome)• Blastomycosis• Factitialdermatitis• Traumaticulceration• Tuberculosisgumma• Hidradenitissuppurativa• Insectbites• PAPAsyndrome• Sporotrichosis• Squamouscellcarcinoma• Venousinsufficiency• Verrucouscarcinoma• Wegenergranulomatosis• Atypicalmycobacterialinfections.

ANTIPHOSPHOLIPID ANTIBODY SYNDROME A33-year-oldfemalepresentedwithblackishdiscolorationoffingers,toesandlowerlegsince1month.Itstartedasmultipleflatreddishlesionsoverthefingertipsandgraduallyprogressedtobecomeconfluent.Thenfingers,toes,feetandevenlowerlegswereinvolved.Patienthadhistoryoffeversince8months.Fivemonthsago,shehadtransientlossofvisionthatlastedfortwodays.Shehadahistoryofthreeconsecutivemiscarriagesall inherfirst trimester.Therewasnohistoryofanyprecipitating factor likedrug intake,smoking,useoforalcontraceptives,anychronicdiseaseorperipheralvasculardisease.

PHYSICAL EXAMINATION Hergeneralconditionwaspoorwithtemperature101°F,pulserate90/minuteandbloodpressure122/78mmHg.Someofhernailsshowedsubungualhemorrhages.

CUTANEOUS EXAMINATION Blackishdiscolorationofallfingers,toes,solesinapatternofsocks(Figs14.6and14.7).

Fig. 14.6 Symmetrical gangrenous changes on fingers and toes

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173 Miscellaneous

Laboratory Investigations Investigationsrevealedanemia,elevatedtotalleukocytecount,plateletcountanderythrocytesedimentationrate.Activatedpartialthromboplastintimeandprothrombintimewerealsomildlyelevated.Peripheralsmearrevealedneutrophilicleukocytosiswithmarkedthrombocytosis.Serumelectrolytes,renalfunctiontests,chestradiography,ultrasoundabdomen,bloodculture,fundusexaminationandechocardiographywerenormal.Antinuclearantibodyprofilewasnegative.Skinbiopsyrevealedvascularthrombosis,dermalhemorrhage,obliteratingendarteritisandepidermalnecrosis.Anticardiolipinantibody IgG, IgMwerepositive inhigh titers.The lupusanticoagulant testwaspositive. Inviewof theclinicaland laboratoryfindings,afinaldiagnosisofAPSwasmade.

Q.6. What is antiphospholipid antibody syndrome? Ans. AntiphospholipidsyndromeorAPSisanautoimmuneconditionwhichischaracterizedbyrecurrentarterialorvenousthrombosisand/orpoorpregnancyoutcomesinthepersistentpresenceofcirculatingantiphospholipidantibodies(aPL).1 Basedonthepresenceorabsenceofunderlyingetiology,itcanbeprimaryorsecondaryAPS.

Conditions associated with APS

Immunosuppressive/Anti-inflammatory Antiproliferative

Common autoimmune or rheumatic diseases with aPL

SLE, Sjögren syndrome, rheumatoid arthritis, autoimmune thrombocytopenic purpura, autoimmune hemolytic anemia, psoriatic arthritis, systemic sclerosis, mixed connective-tissue disease, polymyalgia rheumatic, giant cell arteritis, Behçet syndrome.

Infections Syphilis, hepatitis C infection, HIV infection, human T-cell lymphotrophic virus type 1 infection, malaria, bacterial septicemia.

Drugs Cardiac—procainamide, quinidine, propranolol, hydralazine. Neuroleptic or psychiatric—phenytoin, chlorpromazine. Other—interferon alfa, quinine, amoxicillin

Q.7. What are the clinical manifestations of antiphospholipid syndrome? Ans.• Venous or arterial thrombosis:Involvingthecerebrovascularsystem,coronaryarteries,pulmonaryemboli

orthromboses,hepaticorrenalveins,ocularveinsorarteries• Recurrentabortionsorprematurebirthsorevenpre-eclampsiainpregnancy• Skin disorders:Livedoreticularis,splinterhemorrhages,legulcers,superficialthrombophlebitis,bluetoe

syndrome,vasculitisinvolvingmedium-sizedandsmallvessels(Fig.14.8)• Neurological defects:Migraineheadaches,seizures,multi-infarctdementia• Cardiac abnormalities:Heartmurmur,cardiacvalvevegetations• Blood abnormalities:Thrombocytopenia,hemolyticanemia

Fig. 14.7 Gangrene on tips of fingers

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Q.8. What are the cutaneous manifestations of APS? Ans.• Livedoreticularis• Sneddon’ssyndrome• Skinulcers• Necrotizingvasculitis• Livedoidvasculitis(Figs14.9AandB)• Cutaneousgangrene• Superficialthrombophlebitis• Pseudovasculitic lesions:Nodules,papules,pustules,palmar–plantarerythema• Subungualbleeding• Anetoderma.2-4

Q.9. What are the diagnostic criteria for APS? Ans. In1999anInternationalWorkshopissuedthefirstConsensusStatementonpreliminaryclassificationcriteria fordefiniteAPS.5Theclassificationcriteriawere revised in2006 (revisedSaporrocriteria)anddefiniteAPSwasthepresenceofatleastoneoftheclinicalandoneofthelaboratorycriteriaasoutlinedintablebelow.6

Clinical Criteria

Vascular ThrombosisOne or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ confirmed by objectively validated criteria (imaging or Doppler studies or histopathology), with the exception of superficial venous thrombosis.Pregnancy Morbidity• One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, with

normal fetal morphology documented by ultrasound or by direct examination of the fetus, or• One or more premature births of a morphologically normal neonate at or before the 34th week of gestation because

of severe preeclampsia or eclampsia or severe placental insufficiency, or• Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal

anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded.

Clinical Criteria

Laboratory Criteria1. Lupus anticoagulant present in plasma, on two or more occasions, at least 12 weeks apart, detected according to

the guidelines of the International Society on Thrombosis and Hemostasis (Subcommittee on Lupus Anticoagulants/Antiphospholipid Antibodies).

2. aCL of IgG and/or IgM isotype in serum plasma, present in medium or high titer on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA.

3. Anti-b2GPI of IgG and/or IgM isotype in serum or plasma (titer >99th percentile) on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA according to recommended procedures.

Fig. 14.8 Livedo reticularis

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Q.10. What is the treatment of antiphospholipid syndrome? Ans. Riskfactorsforthrombosisshouldbeidentifiedandremovedorcorrected,forexample,smoking,oralcontraceptives,highbloodpressureorelevatedbloodfats. Treatmentinvolvesprimarythromboprophylaxisandsecondarymanagementofthromboembolism.1. PrimarythromboprophylaxiswithlowdoseaspirinisgiveninallpatientswithAPSandnothrombotic

events.72. Secondarythromboprophylaxisisthetreatmentstartedafteranythromboticepisodetopreventfurther

attacks.LifelongwarfarinkeepingtheINRbetween2and3toavoidhemorrhagiccomplicationsisthecurrentrecommendation.Duringpregnancy,low-molecularweightheparinisused.8

Other antiplatelet agents like dipyridamole, ticlopidine or clopidogrel9 and even intravenousimmunoglobulin10havebeenusedforsecondarythromboprophylaxis.

POLYARTERITIS NODOSA

CHIEF COMPLAINTS• Multiplerawareasinboththelowerlimbsforfourmonths• Blackishdiscolorationoftheleftindexfingerfortwo-monthsand• Constitutionalsymptomslikefever,malaiseandmultiplejointpainsfortwomonths.

Figs 14.9A and B Livedoid vasculitis. (A) Multiple well-defined erosions with surrounding erythematous hue and healed lesions showing wrinking and postinflammatory hypopigmentation on medial aspect of both ankle. (B) Atrophic blanche like lesions on dorsum of feet

A

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HISTORY OF PRESENT ILLNESSAtwentysix-year-oldfemalepresentedwithhistoryoffever,multiplejointpainsalongwithfewpainfulsubcutaneousnoduleswithulcerationsinboththelowerlimbsforfourmonths,andblackishdiscolorationoftheleftindexfingerfortwo-monthduration.Therewasnohistoryofpurpura,Raynaud’sphenomenon,recurrentoralulcers,hairloss,malarrash.Nohistoryofjaundiceortuberculosisinpast.

GENERAL PHYSICAL EXAMINATIONHervitalswerewithinnormallimits.

CUTANEOUS EXAMINATIONFewpunchedoutulcerationswithsurroundinglivedoreticularispredominantlyoverboththelegsanddorsumoffeet andbluishblackdiscolorationofdistalphalanxoftheleftindexfinger.

LABORATORY INVESTIGATIONS Completebloodcountshowedleukocytosis.Therenalfunctionandliverfunctiontestswerenormal.Anti-streptolysinO(ASLO)titer,ESRandCRPwereraised.OtherimmunologicaltestsincludingANA,ANCA,APLA,lupusanticoagulant,rheumatoidfactor,HIV,HBsAgandHCVwerenegative.Thethroatandurinecultureshowednogrowthofbacteria.Theechocardiographyandultrasonographyoftheabdomenandpelviswerenormal.ThedeepskinbiopsytakenfromthesubcutaneousnoduleshowedleukocytoclasticvasculitisofthedermalvesselssuggestiveofcutaneousPAN.Thepatientwastreatedwithmethylprednisolone(threepulsesdosesof750mg/day)followedbyoralprednisoloneof1mg/kg/day.Shealsoreceivedacourseoforalpenicillinforantecedentstreptococcalthroatinfection.Theskinlesionscompletelyhealedoveraperiodoffivemonthswithscarring,andpatientisonregularfollowupsincethen.

Q.11. What is polyarteritis nodosa (PAN)?• Polyarteritis nodosa (cPAN) is a necrotizing inflammation ofmedium to small vessels leading to

microaneurysmformation,aneurysmalrupturewithhemorrhage,thrombosis,and,ultimately,organischemiaorinfarction.

• PAN,likeothervasculitides,affectsmultiplesystemsandhasmultiplemanifestations,althoughitmostcommonlyaffectsskin,joints,peripheralnerves,thegut,andthekidney.11

• CutaneousPANisusuallysaidtobePANaffectingtheskinwithnomajororganinvolvementbutinwhichmildfever,muscle,jointandperipheralinvolvementmayalsooccur.12-14

• ClassicPANorsystemicPANpatientshavemulti-organinvolvementalongwithconstitutionalsymptomslikefeverandweightloss.

Q.12. What is the etiology of PAN?• The exact etiology of CPAN is not known, but immune complexmediated disease plays a role in

etiopathogenesis.• Various infections likeStreptococcus, ParvovirusB19,Mycobacterium,hepatitisvirusesBandCand

noninfectiousconditionslikeconnectivetissuediseases(systemiclupuserythematosus,rheumatoidarthritis),Wegener’s granulomatosis andChurg-Strauss syndromehavebeenassociatedbothwithinitiationandrelapseofthedisease.15-17

Q.13. What are the clinical features of cutaneous polyarteritis nodosa?• ClinicalfeaturesinPANareduetoinflammationofsmallandmediumsizedvesselscausingthrombosis

andfinallyvascularocclusion.Thisprocessoccursinseveralorgansincludingskin.Thediseasehasperiodsofactivityandremission.

• Vasculiticlesionsaremostoftenfoundonthelegsandfeet.Otherareasthatmaybeaffectedincludethearms,trunk,buttocks,andheadandneck.Theyaremostlikelyonpressurepointssuchastheknees,backofthefootandlowerleg.

Themostcommonfindingsare:• Palpablepurpura• Livedoracemosa• Retiformpurpuraand• Punched-outulcers.Painfulsubcutaneousnodules,digitalinfarctsandgangrenearelesscommonlyseeninclassicPAN.

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Q.14. How is cutaneous polyarteritis nodosa diagnosed?• Therearenospecificclinicalandlaboratoryfindings.• Adeepincisionalbiopsy,includingsubcutaneoustissue,isrequiredforthedefinitivediagnosisofthe

disease.Thecharacteristicpathologicfeatureisaleukocytoclasticvasculitisinthesmalltomediumsizedarteriolesofthedeepdermisorhypodermiswithorwithoutassociatedfibrinoidnecrosis.18

• ImmunologicaltestingdoesnothelpinconfirmingthediagnosisofcPAN,however,theyhelptoruleoutothersevereformsofvasculitis.

Q.15. What is the differential diagnosis of PAN?• Cryoglobulinemicvasculitis• Autoimmuneconnectivetissuedisease• MPA• Wegener’sgranulomatosis• Churg-Strausssyndrome• Fibromusculardysplasia• Atherosclerosis• SLE• Cholesterolemboli• Neurofibromatosis• Ehlers–Danlossyndrome• Embolifrominfectiveendocarditisand• Leftatrialmyxoma• Cholesterolemboli• Antiphospholipidsyndrome.

Q.16. What is the treatment for cutaneous polyarteritis nodosa?• MildercasesofcPANaretreatedsymptomaticallywithNSAIDsandacourseofantibioticforantecedent

streptococcalinfection.• Severecasesdefinitelyrequiresystemicsteroidsandevenimmunosuppresiveslikecyclophosphamide,

methotrexateorazathiprine.• Intravenousimmunoglobulinsandbiologics(etarnercept,infliximabandrituximab)havebeentriedwith

goodresults.18,19

• ForhepatitisB–relatedPAN,treatmentconsistscorticosteroidswithantiviralagentsandplasmapheresis.

GRANULOMATOSIS WITH POLYANGIITIS (GPA)/WEGENER’S GRANULOMATOSIS

A55-year-oldmanpresentedwithbloodydischargefromthenosewithnasalobstructionandpain,andareddishswellingneartherightnostrilforfourmonths.Thediseasestartedinsidiouslywithagraduallyincreasingobstructionoftherightsideofnasalcavityandascantyblood-stainedserousdischargefollowedbycrusting.Subsequently,thepatientdevelopedrednessandswellinginsidetheoralcavityoverthehardpalatewhichwascausingdiscomfortwhileswallowingfood.Hehadhistoryoffeverwhichwasassociatedwithweaknesssincepastfewmonths.Nocomplaintofanyspecificsymptomsreferabletoothersystemsapartfromrecurrentupperrespiratorytractinfections.Nofamilyhistoryofasimilarorrelatedillnesswasreported.Nohistoryofsmokingoralcoholaddiction.Nohistoryofdiabetesmellitus(DM),hypertension(HTN),tuberculosis(TB)oranyothermajorsurgicalormedicalillness.Notsignificant(Figs14.10to14.12).

Q.17. How to take relevant history of a case of Wegener’s granulomatosis? Ans. Wegener’sgranulomatosishasaspectrumofclinicalpresentationsthatincludesrecurrentrespiratoryinfectioninadultsandupperandlowerrespiratorytractinvolvmentinchildren.Inaddition,patientsmayreportthefollowingchronic,nonspecificconstitutionalcomplaints:• Fever,nightsweats• Fatigue,lethargy• Lossofappetite• Weightloss.

Ophthalmic manifestations • Conjunctivitis• Episcleritis

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Fig. 14.12 Palatal perforation in case of Wegener’s granulomatosis

Fig. 14.11 Polypoid growth over the palate

Fig. 14.10 Nodule on the right nostril

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• Uveitis• Opticnervevasculitis• Retinalarteryocclusion• Nasolacrimalductocclusion• Proptosis.

Ear, nose, and throat manifestations Chronicsinusitisisthemostcommoninitialcomplaint,occurringin67%ofcases;failuretorespondtoconventionaltreatmentissuggestive.OtherENTmanifestationsareasfollows:• Rhinitis(22%)20• Epistaxis(11%)20

• Collapseofnasalsupport,resultinginsaddlenosedeformity(common)• Serousotitismediaandhearingloss• So-calledstrawberrygingivalhyperplasia• Stridor,possiblyleadingtorespiratorycompromise,fromtrachealorsubglotticgranulomatousmasses.

PULMONARY PulmonaryinvolvementinGPAcanbeasymptomatic,insidiousinonset,orsevereandfulminant.Pulmonarydiseasemaycauseanyofthefollowing:• Pulmonaryinfiltrates(71%)• Cough(34%)• Hemoptysis(18%)• Chestdiscomfort(8%)20• Dyspnea(7%)20• Diffusealveolarhemorrhageduetoalveolarcapillaritis(5–45%)21• Atelectasis,withdullnessonpercussion,decreasedbreathsounds,andcracklesonauscultation.

Musculoskeletal Manifestations

• Myalgias• Arthralgias,usuallypolyarticularandsymmetrical,affectingsmallandmediumjoints• Arthritis,typicallyaffectinglargejoints,butrarelydeforming.

Renal Manifestations

• Crescenticnecrotizingglomerulonephritischaracterizedbyurinarysedimentwithmorethan5RBCsperHPForerythrocytecasts

• Renaldiseaseispresentin17%ofpatientsatinitialdiagnosisandisusuallyasymptomatic22• Renalfailureoccursin11%atpresentation.20

Nervous System Manifestations

Peripheralnervoussystem(PNS)involvementmayoccurinasmanyas67%ofpatients,typicallylaterinthediseasecourse,andincludesthefollowing:• Mononeuritismultiplex• Sensorimotorpolyneuropathy• Cranialnervepalsies.TheCNSmanifestationsincludevasculitisofsmalltomedium–sizedvesselsofthebrainorspinalcordandgranulomatousmassesthatinvolvetheorbit,opticnerve,meninges,orbrain.23

Cutaneous Manifestations

• Cutaneousfindingsarevariableandnonspecificandusuallyaffectthelowerextremities• Palpablepurpuraorskinulcers(45%);20ulcerationsmayresemblepyodermagangrenosum• Petechiae,vesicles,pustules,hemorrhagicbullae,livedoreticularis,digitalnecrosis,subungualsplinter

hemorrhages,andgenitalulcersresemblingsquamouscellcarcinomahavebeenreported.

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Additional systemic findings: • Cardiac:Pericardialrub,myocardialinfarction,orsuddendeath• Gastrointestinal:Abdominalpainmaybepresentwithsplanchnicvasculitis.

Physical Examination

Pulmonary/Respiratory System

Atelectasis:Maybepresent,withexaminationfindingsofdullnessonpercussion,decreasedbreathsounds,andcracklesonauscultation.

Lower respiratory tract involvement:Mayalsoproducesignsofpulmonaryconsolidationand/orpleuraleffusion.

Tracheobronchial disease:Maymanifestashoarseness,cough,dyspnea,stridor,andwheezing.

Subglottic or tracheal stenosis:Mayalsomanifestasstridor;stenosismayprovefatalifuntreated.

Hemoptysis—mayresultfromcavitatedpulmonaryparenchymallesions,DAH,orbronchiectasis.

Cutaneous Examination

Cutaneousfindingsarevariableandnonspecificandusuallyaffectthelowerextremities.Palpablepurpura,papules,subcutaneousnodules,andulcerationsarethemostcommonfindings.Ulcerationsmayresemblepyodermagangrenosum. Petechiae,vesicles,pustules,hemorrhagicbullae,livedoreticularis,digitalnecrosis,subungualsplinterhemorrhages,andulcersresemblingsquamouscellcarcinomahavebeenreported. Generalexamination:Patientsmaybefebrileandappearill. Cardiovascularsystem:Pericardialrubmayoccurwithpericarditis. Gastrointestinalsystem:Abdominalpainmaybepresentwithsplanchnicvasculitis. Musculoskeletalexamination:Large-andmedium-jointarthritis;polyarticular,symmetricalsmall-andmedium-jointarthralgias;andmyalgiasmaybeappreciated. Nervoussystem:Examinationofthenervoussystemmayconfirmapatternofmononeuritismultiplex,polyneuropathy,orcranialnerveparalysis.

Q.18. How to investigate a suspected case of Wegener’s granulomatosis? Ans. Routinelaboratorytestsarenonspecificingranulomatosiswithpolyangiitis(GPA).Elevatedbloodureanitrogen(BUN)andcreatininelevelsmaysignalrenalinvolvement. ESRandC-reactiveprotein(CRP) levelsareelevatedin90%ofpatientswithactiveandgeneralizeddisease.Theymaydecreaseinresponsetotreatment.

Q.19. What is ANCA? Ans. Anti-neutrophilcytoplasmicantibodies(ANCAs)areagroupofautoantibodies,mainlyoftheIgGtype,againstantigensinthecytoplasmofneutrophilgranulocytes(themostcommontypeofwhitebloodcell)andmonocytes. ThediscoveryofANCAswithinneutrophilsinthemajorityofpatientswithGPAsuggestedtheroleofhumoralautoimmunity.GPAisusuallyassociatedwiththepresenceofdiffusestainingcytoplasmicANCA(c-ANCA)directedagainstserineproteinase3antigen(PR3-ANCA),theso-calledWegener autoantigen. TheotherANCAassociatedvasculitis(AAVs)includemicroscopicpolyangiitis,renal-limitedvasculitis,andChurg-Strausssyndrome(allergicgranulomatousangiitis),whicharemorecommonlyassociatedwithperinuclear-stainingANCA(p-ANCA)directedagainstmyeloperoxidase(MPO-ANCA). ApathogenicroleforPR3-ANCAsinGPAhasbeenproposed,becausePR3-ANCAisstronglyassociatedwiththedisease;over90%ofGPApatientshavebeenreportedtohaveANCApositivityduringactivedisease.24LongitudinalobservationshaveindicatedthatrelapseissometimesheraldedbyariseinPR3-ANCAtiters,althoughotherstudiescouldnotconfirmtheseresults.25-27 Thesensitivityof c-ANCA testwas found tobe67%by immunofluorescenceand60%byELISA forpatientswithactivelocalorregionalsymptomatology.28Hence,althoughapositivec-ANCAtestisadjunctiveevidence for thediagnosisofWegenergranulomatosis, theresultmustbeviewed in thecontextof thepatient’sclinicalpictureanddiseaseactivity.

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CT scan: Findings on chest radiography are abnormal in two thirds of adultswithGPA.Themostcommonradiologicfindingsaresingleormultiplenodulesandmasses.Nodulesaretypicallydiffuse,andapproximately50%arecavitated. Diffuse alveolar opacities due to diffuse alveolar hemorrhage (DAH), atelectasis, and obstructivepneumoniacausedbybronchialstenosismayalsobeseen.Findingsoncomputedtomography(CT)scansandhigh-resolutionCT(HRCT)scansincludeconsolidation,patchyordiffuseground-glassopacities,orboth. AdditionalCTscanfindings includestenosesof the larynxor tracheobronchial tree,bronchialwallthickening,bronchiectasis,pleuralthickeningoreffusion,andlymphadenopathy. SinusCTscanningandradiographycanbedoneforfurtherevaluation. Histopathologicalexaminationofbiopsy:stainedwithH&Estainshowedfeaturesofgranulomatousinfiltrationwithfociofnecrosis.Evidenceofvasculitiswithfibrinoiddegenerationwasalsonotedinsomesections. ThediagnosisofGPAisgenerallyconfirmedwithtissuebiopsyfromasiteofactivediseaseandrenalandlungbiopsiesaremostspecificforGPA.However,samplingerrormayoccur,andhistopathologicfindingscanbenonspecific.Tissuediagnosismaynotberequirediftheclinicalgestaltisconvincingandasiteforbiopsyisnotapparentorwouldbetooinvasivetoobtain.Renalbiopsymaybeeasiertoperformthanlungbiopsy.

Q.20. What other differentials should be considered? Ans. OtherconditionstoconsiderinthedifferentialdiagnosisofGPAincludethefollowing:• Cocaineabuse• Acuteglomerulonephritis• Crescenticglomerulonephritis• Diffuseproliferativeglomerulonephritis• Membranoproliferativeglomerulonephritis• Membranousglomerulonephritis• Poststreptococcalglomerulonephritis• Rapidlyprogressiveglomerulonephritis• Lymphomatoidgranulomatosis• Malignancy• Microscopicpolyangiitis• Pneumocystisjirovecipneumonia• Bacterialpneumonia• Fungalpneumonia• Polyarteritisnodosa• Polychondritis• Nasal-typeprimaryNK/T-celllymphoma(formerlyknownaslethalmidlinegranuloma)• Pyodermagangrenosum• Rhinoscleroma• Sarcoidosis• Systemiclupuserythematosus.

Q.21. What is granulomatosis with polyangiitis? Ans. Granulomatosis with polyangiitis (GPA), formerly known asWegener granulomatosis, is a raremultisystem autoimmune disease of unknown etiology. Its hallmark features include necrotizinggranulomatousinflammationandpauci-immunevasculitisinsmall-andmedium-sizedbloodvessels.

Q.22. How will you manage a case of GPA? Ans. Themainstayoftreatmentforgranulomatosiswithpolyangiitis(GPA)isacombinationofcorticosteroidsand cytotoxic agents. Treatment should be tailored to appropriately treat GPAmanifestationswhileminimizinglong-termtoxicitiestothepatient.Initialhigh-doseglucocorticoids(1mg/kg/day)shouldbecontinuedforatleast1month.Dosesshouldnotbereducedtolessthan15mg/daywithinthefirst3months.Thedoseshouldthenbeslowlytaperedtoamaintenancedoseof10mg/dayorlessduringremission. Approximately 90% of patients withGPA respond to cyclophosphamide,with approximately 75%experiencingcompleteremission.However,30–50%ofthosewhoinitiallyrespondexperienceatleastonerelapse,requiringanothercourseoftherapy.Cyclophosphamideisgivenincombinationwithsteroids.

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Therecommendeddailyoraldoseofcyclophosphamideis2mg/kg/day(nottoexceed200mg/day).Pulsed(intravenous)cyclophosphamide(15mg/kgevery2weeksforthefirst3pulses,thenevery3weeksforthenext3–6pulses)isanalternativetodailyoralcyclophosphamide;itresultsinlesscumulativeexposuretocyclophosphamideand,therefore,theoreticallycausesfeweradverseeffects. In 2011, however, theUS Food andDrugAdministration (FDA) approved the use of rituximab (amonoclonal antibody that targets B cells), in combinationwith glucocorticoids, as an alternative tocyclophosphamideforinductionofremissioninAAV(GPAandmicroscopicpolyangiitis).29 Plasmaexchangemaybeconsideredinpatientswithrapidlyprogressiverenaldisease(serumcreatininelevel>5.65mg/dL)inordertopreserverenalfunction.30

SUBCUTANEOUS ZYGOMYCOSIS

CHIEF COMPLAINTS • Lesioninnasalcavitysince18months• Swellingoverlipandfacesince3months• Aggravationofswellingsince2months.

HISTORY OF PRESENT ILLNESS A55-year-oldmalepatienthadhistoryoftraumaintheleftsidenasalcavityoneandhalfyearbackbyvegetativematerial.Followingthisthepatientdevelopedsmallpapularnontender lesionat thesiteoftrauma.Thelesionprogressedinsizeveryslowly.Nocomplaintofbleeding,discharge,respiratorysymptomswereassociated(Figs14.13and14.14). Patientdidnotgiveanyhistoryofpriorsurgery,anyradiationtreatment,orhistory/symptomofcellulitis.Patienthadundergoneexcisionfornasal lesiontwicepreviously fromvariouspractitionersalongwithdebridement.

PHYSICAL EXAMINATION • Cervicallymphadenopathywaspresent.• Systemicexaminationwasnormal.• Head and neck examination:Facialswellingmainlyinvolvingforehead,nose,upperlipandmalarregion

withsmallpoxscars.• Nasalseptalperforationwaspresent.

Q.23. What is zygomycoses? Ans. Thetermsmucormycosisandzygomycosisareusedinterchangeably.Mucormycosisisaninfectioncausedbyfungi intheordersMucoralesandEntomophthorales.Previously,thetermzygomycosiswas

Fig. 14.13 Pre-treatment—grotesque appearance of face in case of phaeohyphomycosis

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usedtodenoteinvasivefungalinfections(IFIs)causedbythefungibelongingtothephylumZygomycota,classZygomycetes,ordersMucoralesandEntomophthorales.TheMucoralesordercontains2families—MucoraceaeandCunninghamellaceae.31 SincethemajorityofhumaninfectionsarecausedbyMucoralesfungi,thetermMucormycosisisnowusedtodesignatethisinfection.32

Q.24. How to investigate a case of deep fungal infection? Ans.Diagnosisrequiresdeepskinbiopsyfromthesubcutaneousmassforhistopathologyandformicroscopyandfungalculture. Culturerevealsfastgrowingfungicharacterizedbyprimitivemostlyaseptatehyphae.Cultureat30°Cconfirmswhichorganismisinvolved:Conidiobolus sp.willgrowwhitetogreywaxycoloniesandBasidiobolus sp.willgrowcreamoryellowwaxycolonies. Careneedstobetakenduringthebiopsynottodamagethefungibecausenon-viableorganismsresultinanegativecultureresult. ChestandabdominalX-raytoseedisseminationishelpful. CTscanswithcontrast,MRIandnasalendoscopytoseetheextentofdiseaseisalsoadvisable. Bronchoscopywithbronchoalveolar lavage and transbronchialbiopsyprovides adequate tissue todiagnosepulmonarymucormycosis. CT-guidedpercutaneouslungbiopsymayalsobebeneficial.33

Histology: Fixed tissue can be stained with hematoxylin and eosin (H & E). Fungal hyphaemay bedemonstratedwithGrocottmethenamine-silverstainorperiodicacid-Schiff(PAS)staining.Thetypicalappearancedemonstratesthefungusasbroad,nonseptatehyphaewithacuteright-anglebranching.

Q.25. Enumerate the various types of mucormycosis? Ans.• Rhinocerebralmucormycosis• Pulmonarymucormycosis• Gastrointestinalmucormycosis• Cutaneousmucormycosis• Disseminatedmucormycosis.

Q.26. What are the risk factors that can be associated with zygomycosis? Ans. Mostpersonswhodevelopmucormycosisareimmunocompromised,although15–20%ofpatientshavenoevidenceofanyunderlyingconditionatthetimeofthediagnosis.34-38Themostcommonriskfactorsincludethefollowing:• Stemcelltransplantation• Poorlycontrolleddiabetesmellitus,eithertype1ortype2• Hematologicmalignancy(e.g.leukemias,lymphomas)

Fig. 14.14 Post-treatment—reduction in the size of the lesion

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• Solidorgantransplants• Steroiduse• Metabolicacidosis• Deferoxaminetherapy• Severeandprolongedneutropenia• Intravenousdruguse• Renalfailure• Peritonealdialysis• Burns• Penetratingtrauma(rare).

Q.27. What differentials should be kept in mind when diagnosing a case of zygomycosis? Ans.• Actinomycosis• Aspergillosis• Brainabscess• Cryptococcosis• Nocardiosis• Pepticulcerdisease• Toxoplasmosis.

Q.29. Management of a case of zygomycosis?39

Managementofzygomycosisisdescribedbelow:

Q.28. What are the other effective treatment measures? Ans.• Itraconazole:400mg/dayfor8–12weeks• Oral KI:500mgTIDfor6–12weeks• Fluconazole• Hyperbaricoxygentherapy• Combinationofantifungals.

Q.30. What is the mortality rate of zygomycosis? Ans. Theoverallmortalityrateassociatedwithzygomycosisisapproximately50%andhasremainedatthislevelforthepast50years.Rhinocerebralzygomycosiscarriesamortalityrateofapproximately85%.Mortalityratesareveryhighbecause,bythetimezygomycosisissuspectedanddiagnosed,ithasfrequentlyspreaddiffuselyandcausedextensivetissuedestruction.However,theriskofmortalityvariesdependingonthecharacteristicsofthehost,thetypeofinfection,thesiteofinfection,andtheuseofsurgicalintervention.Ingeneral,antifungaltherapyandsurgicalmanagementindependentlydecreasethelikelihoodofdeath.36

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