Clinical Correlation with Diagnostic Implications in Dermatology
Brothers
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Prelims.indd 1 02-01-2017 14:02:18 Brothers
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Prelims.indd 2 02-01-2017 14:02:18 Clinical Correlation with Diagnostic Implications in Dermatology
Editor-in-Chief Biju Vasudevan MD (DVL) FRGUHS Associate Professor Department of Dermatology Base Hospital Barrackpore, West Bengal, India
Assistant Editors Shekhar Neema MD (DVL) Ankan Gupta MD (DVL) Assistant Professor Assistant Professor Department of Dermatology Department of Dermatology Command Hospital Christian Medical College and Hospital Kolkata, West Bengal, India BrothersVellore, Tamil Nadu, India Editorial Advisory Committee Rajesh Verma MD (DVL) Manas Chatterjee MD (DVL) Consultant Senior Advisor, Professor and Head Department of Dermatology Department of Dermatology Skin Centre, Base Hospital INHS Asvini Lucknow, Uttar Pradesh, India Mumbai, Maharashtra, India
Foreword Rajan S Grewal
JaypeeThe Health Sciences Publisher New Delhi | London | Panama
Prelims.indd 3 02-01-2017 14:02:18 Jaypee Brothers Medical Publishers (P) Ltd
Headquarters Jaypee Brothers Medical Publishers (P) Ltd 4838/24, Ansari Road, Daryaganj New Delhi 110 002, India Phone: +91-11-43574357 Fax: +91-11-43574314 Email: [email protected] Overseas Offices J.P. Medical Ltd Jaypee-Highlights Medical Publishers Inc 83 Victoria Street, London City of Knowledge, Bld. 235, 2nd Floor, Clayton SW1H 0HW (UK) Panama City, Panama Phone: +44 20 3170 8910 Phone: +1 507-301-0496 Fax: +44 (0)20 3008 6180 Fax: +1 507-301-0499 Email: [email protected] Email: [email protected] Jaypee Brothers Medical Publishers (P) Ltd Jaypee Brothers Medical Publishers (P) Ltd 17/1-B Babar Road, Block-B, Shaymali Bhotahity, Kathmandu Mohammadpur, Dhaka-1207 Nepal Bangladesh Phone: +977-9741283608 Mobile: +08801912003485 Email: [email protected] Email: [email protected] Website: www.jaypeebrothers.com Website: www.jaypeedigital.com © 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. Brothers All rights reserved. No part of this publication may be reproduced, stored or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission in writing of the publishers and author. 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 contraindications. 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. Inquiries for bulk sales may be solicited at: [email protected] Clinical Correlation with Diagnostic Implications in Dermatology First Edition: 2017 ISBN:Jaypee 978-93-86322-66-1 Printed at
Prelims.indd 4 02-01-2017 14:02:18 Dedicated to My family, teachers and patients Brothers
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Prelims.indd 5 02-01-2017 14:02:18 Brothers
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Prelims.indd 6 02-01-2017 14:02:18 Editorial Board
Ananta Khurana MD(DVL) DNB MNAMAS Lakshmi DV MD (DVL) FRGUHS Assistant Professor Senior Resident RML Hospital and PGIMER Department of Dermatology New Delhi, India Bangalore Medical College and Research Institute Bengaluru, Karnataka, India Anju George MD (DVL) DNB FRGUHS Consultant Dermatologist Malcolm Pinto MD (DVL) RMV Hospital Assistant Professor Bengaluru, Karnataka, India Department of Dermatology Yenepoya Medical College Anupam Das MD (DVL) Mangaluru, Karnataka, India Senior Resident Department of Dermatology Sahana M Srinivas DNB DVD FRGUHS (Pediatric KPC Medical College and Hospital Dermatology) Kolkata, West Bengal, India Consultant Pediatric Dermatologist Indira Gandhi Institute of Child Health Anuradha Babu DNB Bengaluru, Karnataka, India Consultant Dermatologist Aster Medcity Hospital Shilpa K MD (DVL) FRGUHS Kochi, Kerala, India AssistantBrothers Professor Department of Dermatology Debdeep Mitra MD (DVL) DNB Bangalore Medical College and Research Institute Graded Specialist Dermatology Bengaluru, Karnataka, India Base Hospital, Delhi Cantt Delhi, India Soumya Jagadeesan MD (DVL) Assistant Professor Divya Gorur K MD (DVL) FRGUHS Department of Dermatology Senior Resident Amrita Institute of Medical Sciences Department of Dermatology Kochi, Kerala, India Bangalore Medical College and Research Institute Bengaluru, Karnataka, India Vinitha Varghese MD (DVL) Associate Professor Indrashis Podder MD (DVL) Department of Dermatology Consultant Dermatologist Amrita Institute of Medical Sciences Kolkata,Jaypee West Bengal, India Kochi, Kerala, India
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Prelims.indd 8 02-01-2017 14:02:18 Contributors
Aarti Sarda MD (DVL) Anagha S MD (DVL) Senior Resident Fellow (Dermatosurgery) KPC Medical College Department of Dermatosurgery Kolkata, West Bengal, India Bangalore Medical College and Research Institute Bengaluru, Karnataka, India Abhishek De MD (DVL) Associate Professor Ananta Khurana MD (DVL) DNB MNAMAS Calcutta National Medical College Assistant Professor Kolkata, West Bengal, India RML Hospital and PGIMER New Delhi, India Aditi Bhagat MD (DVL) Consultant Anju George MD (DVL) DNB FRGUHS Currae Hospital Consultant Dermatologist Thane, Maharashtra, India RMV Hospital Bengaluru, Karnataka, India Ajay Chopra MD (DVL) Head Ankan Gupta MD (DVL) Department of Dermatology Assistant Professor Base Hospital, Delhi Cantt Christian Medical College and Hospital Delhi, India Vellore,Brothers Tamil Nadu, India Akhilesh Shukla MD (DVL) Ankur Talwar MD (DVL) Consultant Dermatologist Assistant Professor Skin and Hair Clinic Department of Dermatology New Delhi, India Hind Institute of Medical Sciences Consultant Dermatologist Akshi Bansal MD (DVL) Talwar Skin Institute Resident Lucknow, Uttar Pradesh, India Department of Dermatology Bangalore Medical College Anupam Das MD (DVL) and Research Institute Senior Resident Bengaluru, Karnataka, India Department of Dermatology KPC Medical College and Hospital Amina Asfiya MD (DVL) Kolkata, West Bengal, India Senior Resident Department of Dermatology Anuradha Babu DNB Yenepoya Medical College Consultant Dermatologist Mangaluru, Karnataka, India Aster Medcity Hospital Kochi, Kerala, India AmitJaypee Bahuguna MD (DVL) Cl Spl (Dermatology and Venereology) Archana S MD (DVL) 155 Base Hospital Associate Consultant Dermatologist Tezpur, Assam, India Bengaluru, Karnataka, India
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Asad Ansari DDVL Deepashree Daulatabad MD (DVL) DNB Senior Resident Senior Resident Calcutta National Medical College Department of Dermatology and STD Kolkata, West Bengal, India University College of Medical Sciences and GTB Hospital Delhi, India Aseem Sharma MD (DVL) MBA Senior Registrar Disha Dabbas MD (DVL) Lokmanya Tilak Municipal Medical College and Cl Spl, Military Hospital Hospital Meerut, Uttar Pradesh, India Mumbai, Maharashtra, India Divya Gorur K MD (DVL) FRGUHS Ashwani Kumar MD (Internal Medicine) Senior Resident Base Hospital, Delhi Cantt Department of Dermatology Delhi, India Bangalore Medical College and Research Institute Bengaluru, Karnataka, India Bhumika S MD FRUGHS Consultant Dermatologist Eswari L MD (DVL) FRGUHS Cuti-Sculpt Clinic Assistant Professor Bengaluru, Karnataka, India Bangalore Medical College and Research Institute Bengaluru, Karnataka, India Bhushan Madke MD (DVL) Associate Professor GK Singh MD (DVL) Jawaharlal Nehru Medical College Cl Spl (Dermatology) Wardha, Maharashtra, India Military Hospital Ahmedabad, Gujarat, India Biju Vasudevan MD (DVL) FRGUHS Associate Professor Gopikrishanan Anjaneyan MD (DVL) DNB Base Hospital AssistantBrothers Professor Barrackpore, West Bengal, India Department of Dermatology Amrita Institute of Medical Sciences Boby Krishna DNB MNAMS DermSurg Fellow Kochi, Kerala, India Consultant Dermatologist and Cosmetologist NIMS Medicity Govind Mittal MD (DVL) FRGUHS Thiruvananthapuram, Kerala, India Consultant Dermatologist and Dermatosurgeon Bengaluru, Karnataka, India Brijesh C MD (DVL) Consultant Dermatologist Harish Shetty MD (DVL) Sanjivani Hospital Senior Consultant Dermatologist Kochi, Kerala, India Kaya Skin Clinic Hyderabad, Telangana, India Debdeep Mitra MD (DVL) DNB Graded Specialist Dermatology Harish Prasad MD (DVL) FRGUHS Base Hospital, Delhi Cantt Consultant Dermatologist Delhi, India Apollo, HCG Cancer Hospital and VITAL Clinic Bengaluru, Karnataka, India Deepak Vasisht MD (DVL) Assistant Professor Indrashis Podder MD (DVL) CommandJaypee Hospital Consultant Dermatologist Pune, Maharashtra, India Kolkata, West Bengal, India
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Ishad Aggarwal MD (DVL) Mary Thomas DNB Consultant Dermatologist Consultant Dermatologist Purnam Medicare Poornima Hospital Kaya Skin Clinic and Pristine Bengaluru, Karnataka, India Kolkata, West Bengal, India Meenaz Khoja DDV Kanhaiya Patidar MBBS Consultant Dermatologist Resident (Dermatology) GMF Ruby Hall Clinic and MMF Joshi Hospital Armed Forces Medical College Pune, Maharashtra, India Pune, Maharashtra, India Nagendra Beniwal DNB Kingshuk Chatterjee DNB MNAMS MRCPS (Glasgow) Assistant Professor Assistant Professor Military Hospital Department of Dermatology Kirkee, Pune, Maharashtra, India Calcutta School of Tropical Medicine Kolkata, West Bengal, India Nagesh TS MD (DVL) DNB Associate Professor Kirti S Jangid MD (DVL) Sapthagiri Institute of Medical Sciences Consultant Dermatologist and Research Center Mumbai, Maharashtra, India Bengaluru, Karnataka, India
Krishna Talukdar MD (DVL) Navya Handa MD (DVL) Associate Professor and In-Charge Head Associate Consultant Jorhat Medical College and Hospital Max Hospital Jorhat, Assam, India New Delhi, India
Lakshmi DV MD (DVL) FRGUHS Neetu Bhari MD (DVL) Senior Resident DepartmentBrothers of Dermatology, Venereology and Department of Dermatology Leprology Bangalore Medical College and Research Institute All India Institute of Medical Sciences Bengaluru, Karnataka, India New Delhi, India
Lekshmipriya B MD (DVL) Nidhi Sharma MD (DVL) Graded Specialist (Derm and Ven) Senior Resident 151 Base Hospital Calcutta National Medical College Guwahati, Assam, India Kolkata, West Bengal, India
Maheshwari NS DVD FRGUHS (Pediatric Niharika Lal MD (DVL) Dermatology) Assistant Professor Consultant Dermatologist Department of Dermatology Bengaluru, Karnataka, India ESI-PGIMSR and ESIC Medical College Kolkata, West Bengal India Malcolm Pinto MD (DVL) Assistant Professor Nikita Lodha DNB Department of Dermatology Consultant Dermatologist Yenepoya Medical College Clear Skin Clinic Mangaluru,Jaypee Karnataka, India Mumbai, Maharashtra, India
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Nirmal Krishna B MD (DVL) Rajesh Kumar Mandal MD (DVL) Assistant Professor Assistant Professor Department of Dermatology Department of Dermatology Christian Medical College North Bengal Medical College Vellore, Tamil Nadu, India Darjeeling, West Bengal, India Pallavi Ailawadi MD (DVL) DNB Rajeshwari Dabbas MD (DVL) Senior Resident Assistant Professor Maulana Azad Medical College Air Force Command Hospital and Lok Nayak Hospital Bengaluru, Karnataka, India New Delhi, India Resham Vasani MD (DVL) DNB Parul Thakur MD (DVL) DNB Consultant Dermatologist Associate Dermatologist Anand Polyclinic Alok Clinic Mumbai, Maharashtra, India Panvel, Maharashtra, India Ruby Venugopal MD (DVL) Piyush Kumar MD (DVL) Dermatologist Associate Professor Command Hospital (Western Command) Katihar Medical College Chandigarh, India Katihar, Bihar, India Sacchidanand S MD (DVL) DHA FRCP Preema Sinha MD (DVL) Director Assistant Professor Department of Medical Education Department of Dermatology Government of Karnataka Armed Forces Medical College Karnataka, India Pune, Maharashtra, India Sahana M Srinivas DNB DVD FRGUHS (Pediatric Priyadarshini Kharge DDVL FRGUHS Dermatology)Brothers Consultant Pediatric Dermatologist Consultant Pediatric Dermatologist Department of Dermatology Indira Gandhi Institute of Child Health Bangalore Medical College and Research Institute Bangaluru, Karnataka, India Bengaluru, Karnataka, India Saloni Katoch MD (DVL) Priyanka Borde Bisht DNB (DV) Consultant Dermatologist Consultant Dermatologist Columbia Asia Hospital Skin City Clinic Bengaluru, Karnataka, India Pune, Maharashtra, India Samipa S Mukherjee DDV DDVL FRGUHS Rahul Arora MD (DVL) Consultant Pediatric Dermatologist and Senior Resident Dermatotrichologist University College of Medical Sciences Cutis Academy of Cutaneous Sciences New Delhi, India Bengaluru, Karnataka, India Rajan S Grewal VSM Samujjala Deb MD (DVL) FRGUHS Commandant—Command Hospital (Southern St. John’s Medical College and Hospital Command) Bengaluru, Karnataka, India Professor DepartmentJaypee of Dermatology Sanjay Singh MD (DVL) Armed Forces Medical College and Department of Dermatology, Venereology and Leprology Command Hospital (Southern Command) All India Institute of Medical Sciences Pune, Maharashtra, India New Delhi, India
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Sankeerth V MD FRGUHS Soumya Jagadeesan MD (DVL) Consultant Dermatologist and Cosmetologist Assistant Professor Apollo Spectre Hospitals Department of Dermatology Bengaluru, Karnataka, India Amrita Institute of Medical Sciences Kochi, Kerala, India Savitha AS MD (DVL) Assistant Professor Sriharsha Talabhukta MD (DVL) Department of Dermatology Assistant Professor Sapthagiri Institute of Medical Sciences Gayatri Vidya Parishad Healthcare and Bengaluru, Karnataka, India Medical Technology Shekhar Neema MD (DVL) Visakhapatnam, Andhra Pradesh, India Assistant Professor Sujala S Aradhya MD FRGUHS Command Hospital Consultant Dermatologist and Kolkata, West Bengal, India Dermatosurgeon Sherina Laskar MD DNB MSc Sujala Polyclinic and Laboratory Resident Physician Bengaluru, Karnataka, India Department of Dermatology Sukesh Mathapathi MD (DVL) DNB Gauhati Medical College Consultant Dermatotrichologist and Guwahati, Assam, India Hair Transplant Surgeon Shilpa Garg DNB (DV) Bengaluru, Karnataka, India Consultant Dermatologist Sumit Gupta MD (DVL) Sir Ganga Ram Hospital Consultant (Dermatology) New Delhi, India New Delhi, India Shilpa K MD (DVL) FRGUHS SuneilBrothers Gandhi MD (DVL) DNB Assistant Professor Department of Dermatology Consultant Dermatologist Bangalore Medical College and Research Institute Reviva Skin Clinic Bengaluru, Karnataka, India Surat, Gujarat, India
Shreyaswini Pujary DNB Surajit Gorai MD (DVL) Consultant Dermatologist Consultant (Dermatology) Kaya Skin Clinic Bardhaman, West Bengal, India Mumbai, Maharashtra, India Tanmay Padhi MD (DVL) Shruthi C MD (DVL) Assistant Professor Department of Dermatosurgery Veer Surendra Sai Institute of Bangalore Medical College and Research Institute Medical Sciences and Research Bengaluru, Karnataka, India Sambalpur, Odisha, India
Siddharth Sonthalia MD (DVL) DNB Tanumay Raychaudhury MD (DVL) Director and Consultant Consultant Skinnocence – The Skin Clinic Tata Medical Centre Gurugram,Jaypee Haryana, India Kolkata, West Bengal, India
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Tarang Goyal MD (DVL) Vishal Sharma MD (DVL) DNB Professor and Head Cl Spl Department of Dermatology, Venereology Command Hospital (Northern Command) and Leprosy Udhampur, Jammu & Kashmir, India Muzaffarnagar Medical College Muzaffarnagar, Uttar Pradesh, India
Vinitha Varghese MD (DVL) Associate Professor Department of Dermatology Amrita Institute of Medical Sciences Kochi, Kerala, India
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Prelims.indd 14 02-01-2017 14:02:19 Foreword
The fascinating panoramic canvas of dermatology portrayed through its myriad clinical presentations puts the oft-quoted adage ‘A picture is worth a thousand words’; in the shade. In the era of exponential advancements in technology driven, skill-based dermatology, with its alluring commercial bottom line, this unique book is a refreshingly informative achievement. It fortifies the foundations of conventional dermatology; which one speculates, is not the flavor of the season amongst the young achievers, who seem enamoured by the reward-oriented conveyor belt of procedure-based interventions. This book elucidates clinical approaches to a multitude of dermatological disorders with their dilemmas and diagnostic implications. We are sanguine that it will be a boon for clinicians, and the entire medical fraternity, including postgraduates, academicians, practitioners; and even MBBS students. The contemporary chapters with their educative scientific contents will be a value addition in our comprehension of pragmatic approaches to patients who present with various dermatologic, genitourinary and leprology conditions. It shall also help the readers to navigate through the common differential diagnosis, and customize the investigations, to arrive at the accurate diagnosis, in the best interests of our patients, who ought to be the ultimate beneficiaries. This book with its unique format, provides cohesive clinicodiagnostic insights to enhance our perspective as a quantum force multiplier. It encompasses recent advances in the clinical, investigational, diagnostic and therapeutic arenas, thereby adding substantially to the knowledge quotient. The format also ensures simplicity of learning in a reader-friendly manner. We are confident that this book will be of immense valueBrothers in the field of dermatology. Its uncomplicated and informative contents should make it a ‘must have’ for students, faculty, practitioners and healthcare professionals.
Rajan S Grewal VSM MD Commandant—Command Hospital (Southern Command) Professor Department of Dermatology Armed Forces Medical College and Command Hospital (Southern Command) Pune, Maharashtra, India Jaypee
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Prelims.indd 16 02-01-2017 14:02:19 Preface
The idea of Clinical Correlation with Diagnostic Implications in Dermatology was born due to the felt need for a book which would help everyone dealing with dermatological manifestations to decide their approach to these conditions. Similar titles do exist in other specialties, but there was a vacuum when it came to books with an approach to dermatological manifestations. This is a dream project meant primarily for postgraduates in dermatology to help them in their approach to various dermatological conditions and also help them in preparing for their cases in practical examinations. It is however equally beneficial for practising dermatologists, undergraduates and other specialists to define their approach when they come across any dermatological manifestation. Every topic has been chosen keeping the relevance to the above matter and the approach, both clinical and investigational is complemented by relevant images. Common dermatological disorders, interesting and exam- oriented topics are presented with an algorithmic kind of approach. Multiple questions and answers in a logical fashion leading to a clinical diagnosis and relevant investigations to confirm the diagnosis, form the pattern for all chapters. The contributors have been chosen from all over the country with utmost care and they are all young academicians who are designing such approaches regularly and who are likely to be the real guiding forces in dermatology in the near future. Section editors of different sections have reviewed the chapters and have made their contributions to both the text and images. The book aims to be a benchmark for approach towardsBrothers dermatological disorders. The logically flowing question-and-answer format following a clinical case presentation lends a unique approach towards managing the various conditions. All members who have contributed to the book have been duly acknowledged. The book has 25 sections with 129 chapters. We hope the sincere efforts of the entire editorial team will live up to the expectations of all concerned.
Biju Vasudevan
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Prelims.indd 18 02-01-2017 14:02:19 Acknowledgments
All faculty members, residents and staff of Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, and Bangalore Medical College, Bengaluru, Karnataka, India.
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Prelims.indd 19 02-01-2017 14:02:19 Brothers
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Prelims.indd 20 02-01-2017 14:02:19 Contents
Section 1: Drug Reactions 12. Melanocytic Nevus 52 GK Singh Savitha AS 1. Toxic Epidermal Necrolysis 3 Section 4: Pediatric Dermatology Asad Ansari, Shekhar Neema Sahana M Srinivas 2. Drug Hypersensitivity Syndrome 7 Anupam Das, Biju Vasudevan 13. Pustular Lesions in Neonates 59 Divya Gorur K, Amina Asafiya 3. Acute Generalized Exanthematous Pustulosis 10 14. Neonatal Erythroderma 63 Soumya Jagadeesan Lakshmi DV 4. Fixed Drug Eruption 14 15. Epidermolysis Bullosa 68 Sumit Gupta Sahana M Srinivas 16. Ichthyosis 73 Section 2: Bullous Disorders Samipa S Mukherjee Soumya Jagadeesan 17. Palmoplantar Keratoderma 77 5. Pemphigus Vulgaris 21 Resham Vasani, Shreyaswini Pujary Vishal Sharma, Biju Vasudevan 18.Brothers Staphylococcal Scalded Skin Syndrome 81 6. Pemphigus Foliaceus 25 Saloni Katoch Deepashree Daulatabad 19. Kawasaki’s Disease 85 7. Subepidermal Blistering Disorders 29 Priyadarshini Kharge Mary Thomas 20. Gianotti-Crosti Syndrome (Papular Acrodermatitis of Childhood) 88 Section 3: Nevi and Vascular Sahana M Srinivas Malformations/Tumors 21. Viral Exanthem 91 Deepashree Daulatabad Sahana M Srinivas 8. Verrucous Epidermal Nevus 37 Bhushan Madke Section 5: Disorders of 9. Nevus Sebaceous 40 Appendages (Hair and Nail) Kingshuk Chatterjee Lakshmi DV 10. Vascular Malformation 42 22. Alopecia Areata 97 Sahana M Srinivas Shilpa K 11. Angiokeratoma 49 23. Androgenetic Alopecia 102 JaypeeNavya Handa Sukesh Mathapathi
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24. Female Pattern Hair Loss 107 39. Porphyria 178 Deepak Vasisht, Kanhaiya Patidar Ananta Khurana 25. Trichotillomania 111 Pallavi Ailawadi Section 9: Sexually Transmitted Disorders 26. Cicatricial Alopecia 115 Malcolm Pinto Savitha AS 40. Genital Ulcer 185 27. Trachyonychia 121 Ankur Talwar Nirmal Krishna B 41. Inguinal Bubo 188 Samujjala Deb Section 6: Disorders of Sweat 42. Secondary Syphilis 192 and Sebaceous Glands Ankan Gupta Surajit Gorai 43. Urethral Discharge 196 Malcolm Pinto 28. Hyperhidrosis 127 Eswari L 44. Vaginal Discharge 201 Anju George 29. Hidradenitis Suppurativa 131 Nagesh TS 45. Genital Warts 207 Amina Asafiya 30. Acne 134 Shilpa Garg, Priyanka Borde Bisht 46. Human Immunodeficiency Virus Infection 211 Shekhar Neema 31. Rosacea 138 Saloni Katoch Section 10: Leprosy Section 7: Vascular Disorders Tanmay Padhi Brothers47. Borderline Tuberculoid Leprosy 219 Ananta Khurana Akshi Bansal 32. Vasculitis 145 48. Lepromatous Leprosy 223 Resham Vasani, Shreyaswini Pujary, Biju Vasudevan Mary Thomas 33. Livedoid Vasculopathy 151 49. Type 2 Lepra Reaction 227 Aseem Sharma, Biju Vasudevan Tanmay Padhi 34. Behcet’s Disease 155 50. Type 1 Lepra Reaction 231 Aditi Bhagat, Biju Vasudevan Harish Shetty, Mary Thomas 35. Sweet Syndrome 159 Disha Dabbas Section 11: Pigmentary Disorders 36. Leg Ulcer 163 Divya Gorur K Neetu Bhari, Sanjay Singh 51. Melasma 237 Siddharth Sonthalia, Deepashree Daulatabad Section 8: Photosensitive Disorders 52. Diffuse Facial Pigmentation 241 Debdeep Mitra Vinitha Varghese 37. Polymorphic Light Eruption 171 53. Reticulate Pigmentary Disorders 245 JaypeeAnju George Samipa S Mukherjee 38. Xeroderma Pigmentosum 174 54. Pigmented Purpuric Dermatoses 248 Maheshwari NS Indrashis Podder, Biju Vasudevan
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55. Vitiligo 251 70. Discoid Eczema 321 Debdeep Mitra, Ajay Chopra, Krishna Talukdar Preema Sinha 71. Airborne Contact Dermatitis 324 Section 12: Psoriasis and Related Disorders Rajesh Kumar Mandal Anupam Das 72. Blistering Beetle Dermatitis 327 56. Psoriasis Vulgaris 259 Ishad Aggarwal, Anju George Bhushan Madke 57. Psoriatic Arthritis 263 Section 15: Skin Tumors Rajeshwari Dabbas, Ashwani Kumar Indrashis Podder 58. Pustular Psoriasis 267 73. Bowen’s Disease 333 Vinitha Varghese Navya Handa 59. Reactive Arthritis 271 74. Squamous Cell Carcinoma 336 Suneil Gandhi Indrashis Podder Section 13: Other Keratinization Disorders 75. Basal Cell Carcinoma 341 Tarang Goyal Saloni Katoch 76. Melanoma 344 60. Pityriasis Rosea 279 Indrashis Podder Sriharsha Talabhukta, Anupam Das 77. Cutaneous Lymphoma 348 61. Porokeratosis 283 Tanumay Raychaudhury, Navya Handa Aarti Sarda, Abhishek De, Saloni Katoch 62. Perforating Dermatoses 286 Section 16: Skin and Systemic Disease Indrashis Podder Shilpa K 63. Pityriasis Rubra Pilaris 290 Brothers 78. Neurofibromatosis 355 Ruby Venugopal, Biju Vasudevan Lakshmi DV 64. Darier’s Disease 294 79. Tuberous Sclerosis 360 Anuradha Babu Kirti S Jangid 65. Follicular Keratotic Disorders 298 Anupam Das 80. Systemic Amyloidosis 365 Anuradha Babu 66. Erythroderma 301 Ankan Gupta 81. Lipoid Proteinosis 369 Tanumay Raychaudhury, Navya Handa Section 14: Eczemas 82. Xanthomatosis 372 Anju George Nagendra Beniwal 67. Seborrheic Dermatitis 307 83. Endogenous Ochronosis 376 Saloni Katoch Nikita Lodha 68. Atopic Dermatitis 312 84. Calcinosis Cutis 380 Tarang Goyal, Samipa S Mukherjee Nikita Lodha 69. Hand Eczema 317 85. Mastocytosis 384 JaypeeBoby Krishna Debdeep Mitra, Ajay Chopra
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Section 17: Infections-1 (Bacterial, 102. Post-Kala-Azar Dermal Leishmaniasis 447 Mycobacterial and Fungal) Piyush Kumar Lakshmi DV Section 19: Mucosal Disorders 86. Pyoderma (Folliculitis) 389 Anupam Das Ananta Khurana 103. White Lesions in Oral Cavity 453 87. Erythrasma 394 Harish Prasad Meenaz Khoja 104. Oral Lichen Planus 457 88. Pitted Keratolysis 397 Niharika Lal Sujala S Aradhya, Sacchidanand S 105. Oral Ulcer 460 89. Actinomycosis 400 Anupam Das Piyush Kumar 106. Red Lesion on Penis 464 90. Lupus Vulgaris 403 Amit Bahuguna Soumya Jagadeesan 91. Tinea Corporis 406 Section 20: Nutritional Disorders Lakshmi DV Priyadarshini Kharge 92. Tinea Pedis 410 107. Acrodermatitis Enteropathica 469 Rahul Arora Bhumika S 93. Onychomycosis 413 108. Pellagra 473 Aarti Sarda, Abhishek De, Lakshmi DV Parul Thakur 94. Pityriasis Versicolor 418 Sankeerth V Section 21: Autoimmune 95. Mycetoma Foot 421 BrothersConnective Tissue Disorders Shilpa K Nirmal Krishna B 109. Systemic Lupus Erythematosus 479 Section 18: Infections-2 Govind Mittal (Viral and Parasitic) 110. Discoid Lupus Erythematosus 485 Ruby Venugopal Malcolm Pinto, Akshi Bansal 96. Chickenpox 427 111. Scleroderma 488 Kirti S Jangid Shekhar Neema 97. Herpes Zoster 431 112. Morphea 492 Ankur Talwar Lekshmipriya B 98. Herpes Labialis 434 113. Dermatomyositis 496 Sankeerth V Nirmal Krishna B 99. Molluscum Contagiosum 437 Shruthi C, Sujala S Aradhya Section 22: Connective Tissue Disorders 100. Eczema Herpeticum or Kaposi Varicelliform Navya Handa Eruption 440 114. Lichen Sclerosus 503 JaypeeArchana S Navya Handa 101. Cutaneous Leishmaniasis 443 115. Atrophoderma and Anetoderma 506 Piyush Kumar Rajesh Kumar Mondal
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116. Keloids and Hypertrophic Scars 509 Section 25: Miscellaneous Disorders Nidhi Sharma, Shekhar Neema Harish Prasad Section 23: Granulomatous Disorders 123. Lichen Planus 543 Indrashis Podder Meenaz Khoja 124. Annular Erythema 547 117. Sarcoidosis 515 Akhilesh Shukla Ishad Aggarwal 125. Erythema Multiforme 551 118. Granuloma Annulare 519 Anju George Meenaz Khoja 126. Pruritic Urticarial Papules and Plaques of Pregnancy 555 Section 24: Pruritic Conditions Indrashis Podder Gopikrishnan Anjaneyan 127. Erythema Nodosum 559 119. Generalized Pruritus 525 Sherina Laskar Brijesh C 128. Cold-Related Conditions: Chilblains, 120. Scabies 529 Frostbite and Raynaud’s Phenomenon 562 Gopikrishnan Anjaneyan, Brijesh C GK Singh, RS Grewal 121. Urticaria 532 129. Corns and Callosities 570 Shruthi C Anagha S 122. Angioedema 537 Gopikrishnan Anjaneyan IndexBrothers 575
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Prelims.indd 25 02-01-2017 14:02:19 Chapter 10
Vascular Malformation
Sahana M Srinivas
Case study 1 Capillary Hemangioma (Infantile Hemangioma) Presenting Complaints Doctor my child is having red skin lesions on the face noticed after 2 weeks of birth and is increasing in size gradually from past 6 months. Setting Outpatient department. History of Presenting Illness An 8-month-old male child presented with complaints of asymptomaticBrothers red skin lesions on left forehead, left eyelid and left cheek from 2 weeks of age. Mother gives history that the skin lesions are increasing in size from past 6 months. She also complains that her child is not able to open her left eye completely. Child is otherwise normal with no similar lesions elsewhere on the body. There is no history of eye abnormalities or suggestive of any neurological involvement. General Physical Examination and Systemic Examination No abnormality detected. Dermatological Examination • Well-defined bright red dome-shaped papules coalescing to form large plaque with lobulated surface measuring 10 cm × 6 cm present on the left forehead extending to left upper eyelid, left lower eyelid and left cheek. • Noncompressible on palpation, partially blanching on pressure. • Oral mucosa, ocular mucosa, palms, soles and scalp—within normal limits.
Q1. Based on the history and clinical examination, Ans: a. Infantile hemangioma. Diagnosis is made on what is the most likely diagnosis? clinical grounds. The characteristic natural history a. Infantile hemangioma (segmental type) of hemangioma (that is lesions noticeable after b. Port-wine stain birth with slowly proliferating phase for the next c. Epidermal verrucous nevus 6 months) along with the morphology and site Jaypeed. Congenital hemangioma suggests a diagnosis of infantile hemangioma.
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Q2. Enumerate the clinical features of infantile hemangioma? Ans: Clinical features are: yy Infantile hemangioma or capillary hemangioma is the most common benign soft tissue tumor of childhood. yy Have distinctive life cycle, characterized by proliferative phase for 9–12 months, and occasionally till 18–24 months in early infancy, followed by an involutional phase leading to complete spontaneous regression in most children, leaving residual fibrous tissue. yy They anc occur on any part of the body as solitary or multiple lesions, but most commonly involve the head and neck regions. Facial hemangiomas Fig. 1: Superficial type of hemangioma on the scalp develop along the four facial segments. Q3. How do you classify infantile hemangiomas? Ans: Infantile hemangiomas can be classified as: yy Hemangiomas may occur as superficial, deep or mixed type. yy Superficial hemangiomas present as bright red, dome shaped to lobulated papules, plaques, nodules (Fig. 1). They are rubbery in consistency, noncompressible on palpation and partially blanch with pressure. yy Deep hemangiomas present as subcutaneous partially compressible nodules and tumors often with an overlying blue hue, prominent venous Brothers network or telangiectasias (Fig. 2). yy Combined hemangiomas have both superficial bright red component and deeper blue nodular Fig. 2: Deep hemangioma with telangiectasias on the right ala of component (Fig. 3). nose Based on the anatomical site, it can be classified into localized (77%), segmental (18%) (Fig. 4) and multifocal type (5%). Q4. What is the estimated rate of complete involution in infantile hemangiomas? a. 30% by 3 years b. 50% by 5 years c. 70% by 7 years d. >90% by 9–10 years e. All of the above are correct Ans: e. All of the above. Infantile hemangioma will not involute with normal skin, but with residual changes like telangiectasias, atrophy, scarring or fibrofatty tissue.
Q5. JaypeeEnumerate the risk factors associated with heman Combined hemangioma on the right cheek with superficial Fig. 3: gioma situated on functionally threatening sites? and deep component
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Fig. 4: Segmental hemangioma Fig. 5: Ulcerated hemangioma Ans: The risk factors associated with hemangiomas located yy Ulcerations (Fig. 5) on different anatomical sites are listed in Table 1. yy Bleeding Q6. What are complications associated with infantile yy Infections hemangiomas? yy High output cardiac failure yy Ocular complications Ans: Complications depend on the type and location of hemangiomas. Common complications associated yy Airway obstructions Site dependent with infantile hemangioma are: yy Interference with feeding complications yy Obstruction of external auditory meatus. Table 1: Location and morphology of infantile Q7. What are the systems involved in infantile hem hemangioma and associated risks angiomas? Anatomic location/ Associated risk Ans: In 10–25% of the cases, multiple lesions are present, Morphology Brothersand may indicate systemic involvement. Evaluation Large segmental facial PHACES* syndrome for hepatic involvement is recommended in infants Nasal tip, ear, large facial Permanent scarring and with 5 or more lesions. Visceral hemangiomatosis (especially with prominent disfigurement may occur without cutaneous lesions as well. dermal component) Visceral hemangiomas may rarely involve lungs, Periorbital and retrobulbar Ocular axis occlusion, gastrointestinal tract (GIT), central nervous system astigmatism, amblyopia, tear- (CNS), intestine, brain, eyes, spleen, oral mucosa and duct occlusion renal systems. Segmental mandibular (beard Airway hemangioma area) and central neck Q8. What are the syndromes associated with infantile Perioral, lips Ulceration, disfigurement hemangiomas? Lumbosacral hemangiomas Tethered spinal cord, genital a. PHACES syndrome and vesicorenal anomalies b. LUMBAR syndrome (imperforate anus, abnormal c. PELVIS syndrome genitalia, skin tags, bony sacral anomalies) d. SACRAL syndrome e. All of the above Perianal, axilla, neck Ulceration, bleeding, infection, scarring Ans: d. All of the above. Multiple hemangiomas Hepatic involvement with risk Q9. When should you consider hemangiomas for of congestive heart failure and complete evaluation and investigations? hypothyroidism Ans: • Many of the hemangiomas that are single and *PHACESJaypee syndrome: P, posterior fossa and other brain malformations; H, are not life-threatening do not require any hemangioma; A, arterial anomalies typically of the aortic branches (aortic branch vessels); C, cardiac defects and coarctation of the aorta; E, eye anomalies; evaluation. They resolve spontaneously without S, sternal defects and supraumbilical raphe. any treatment.
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yy Hemangiomas that should be considered for yy Infantile myofibromatosis complete evaluation and aggressive therapy yy Venous and lymphatic malformations. are those that are life-threatening or function- threatening, like hemangiomas present on Q12. How do you differentiate rapidly involuting and periocular region, nasal tip, ear (extensive), lips, noninvoluting congenital hemangiomas? genitalia or perineum, large facial hemangiomas, Ans: Differences between rapidly involuting congenital hemangiomas present on beard area, lumbosacral, hemangioma (RICH) and noninvoluting congenital multiple and segmental hemangiomas. hemangiomas (NICH) are listed in Table 2. Q10. What are the investigations to be done while Q13. What are the goals of management of infantile evaluating an infantile hemangioma? hemangioma? Ans: Infantile hemangiomas generally require no Ans: Goals of management of infantile hemangioma are: investigations, as the diagnosis is clinically evident. yy Prevention or reversal of life-threatening or In certain cases (as outlined above), the following are function-threatening complications. required: yy Prevention of permanent disfigurement, yy Doppler ultrasound to differentiate deep hem diminution of psychosocial stress for the patient angiomas with vascular malformation. and family. yy Magnetic resonance imaging, computed tomo yy Avoidance of aggressive or detrimental graphy scan and radiography imaging. interventions in lesions that may have excellent yy Ophthalmological evaluation if eyelid involvement prognosis without treatment. is present. yy Prevention or treatment of ulceration to lower yy Complete blood count, liver function test, scarring, infection and pain. coagulation studies, stool examination, Q14. What is the first line of therapy in hemangiomas echocardiography, ECG, abdominal and CNS which are life-threatening and function imaging. threatening? yy Direct laryngoscopy for airway involvement. Ans: First line of therapy includes: Q11. What are the other differential diagnoses to be yy Propranolol, a beta blocker, is the first-line therapy considered for infantile hemangioma? for hemangiomas requiring treatment. Brothersyy It is given at the dosage of 2–3 mg/kg body weight Ans: Other differential diagnoses to be considered are: 8 hourly until clinically evident results are achieved, yy Congenital hemangiomas (rapidly involuting and and then maintained on 0.5–1 mg/kg body weight. noninvoluting congenital hemangioma) Studies have shown long-term usage of around yy Capillary malformation (Port-wine stain) 9–12 months would prevent recurrences. yy Kaposiform hemangioendothelioma yy Contraindicated in children with bradycardia, yy Tufted angiomas bronchial asthma, heart failure, hypotension, yy Pyogenic granuloma hypoglycemia, CNS vascular anomalies.
Table 2: Differences between RICH and NICH Infantile hemangioma RICH NICH Absent, precursor or present at birth Fully developed at birth Fully developed at birth Rapid postnatal proliferation Intrauterine proliferation Proportionate growth Slow spontaneous involution Rapid involution after the first year Do not involute spontaneously More common in girls Equal prevalence or slightly more common Slightly more common in boys in girls Lobular endothelial proliferation during Capillary lobules within fibrotic stroma Lobules of small, thin-walled vessels with proliferative phase; fibrofatty tissue during containing thin-walled vessels, hemosiderin large central vessel; dilated, dysplastic veins involution between lobules; “hobnailed” endothelial Jaypee cells lining intralobular vessels GLUT1-positive, Lewis Y antigen-positive GLUT1-negative, Lewis Y antigen-negative GLUT1-negative
Abbreviations: GLUT1, glucose transporter 1
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yy Pre-evaluation of blood pressure, ECG, Ans: f. All of the above. echocardiography, chest X-ray and blood sugars Active nonintervention: is necessary. yy Education of parents about the natural course of yy Adverse reactions include hypoglycemia, infantile hemangioma hypotension, bradycardia, sleep disruption, yy Regular follow up to assess the course of the lesion acrocyanosis and diarrhea. yy To address emotional and psychological issues Q15. What are the other treatment modalities for raised due to cosmetic disfigurement. infantile hemangiomas? Supportive therapy: a. Active nonintervention yy Bland emollients b. Supportive therapy yy Saline soaks and topical antibiotics for ulcerated lesions c. Topical—corticosteroids, intralesional steroid, timolol, imiquimod yy Oral antibiotics for secondary infection. d. Systemic treatment—oral propranolol, The different treatment modalities are listed in Table corticosteroids, interferon-alpha, vincristine, 3. cyclophosphamide and bleomycin e. Surgical modalities—excision, laser therapy, sclerotherapy, embolization and radiotherapy f. All of the above
Table 3: Different treatment modalities Therapeutic options Indications Topical corticosteroids Superficial hemangioma or precursor lesions in attempt to prevent/alter proliferative phase Intralesional corticosteroids Accessible sites such as the lip, cheek, nasal tip glabellar and periocular areas Imiquimod 5% cream Superficial hemangioma in “cosmetically sensitive” areas Timolol 0.5% lotion Single, superficial hemangiomas with noBrothers deep component <4–5 cm Systemic corticosteroids (1–4 Life or function-threatening hemangiomas—airway, hepatic, periorbital, hemangiomas with persistent mg/kg/day) ulceration or at sites where significant disfigurement is likely Vincristine Life or function-threatening hemangiomas resistant to treatment with systemic steroids (1–1.5 mg/m2/week) Interferon-α2a, b Life or function-threatening infantile hemangioma in older children (particularly in infants >1 year of age (3 µm/m2/day or QOD) when risk of neurotoxicity is lower) Surgical excision/debulking Function-threatening infantile hemangioma (periorbital), hemangioma where scarring/anetoderma are expected (large, pedunculated or painfully ulcerated hemangioma) Pulsed dye laser (595 nm) Ulcerated hemangiomas, superficial hemangiomas Bare-fiber (Nd:YAG) Deep hemangioma in life- or function-threatening areas
Abbreviation: Nd:YAG, neodymium-doped yttrium-aluminum garnet Jaypee
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Case study 2 Port-wine Stain Presenting Complaints Doctor my child is having red skin lesions on the face since birth, the lesions are nonprogressive in nature. Setting Dermatology outpatient department. History of Presenting Illness A 5-year-old female child presented with complaints of asymptomatic red skin lesions on left forehead, left upper eyelid, left cheek and left side of upper lip from birth. Mother gives history that the skin lesions are not increasing in size and have been persistent as it is from birth. There is history of seizures in the child for which the child is on anticonvulsants from past 4 years. Child is otherwise normal with no similar lesions elsewhere on the body. There is no history suggestive of eye or hearing abnormalities. General Physical Examination Child is conscious and alert. Child has normal development with height and weight at the 50th percentile with normal progress on her growth curve. General physical examination and vitals are normal. There is no facial asymmetry. Systemic Examination No abnormality detected. Dermatological Examination • Well-defined dark red macule measuring 12 cm × 8 cm present on the left forehead extending to left upper eyelid, left lower eyelid, left cheek and left upper lip not crossing the midline. • Smooth on palpation and does not blanch on pressure. • Oral mucosa, ocular mucosa, palms, soles and scalp—withinBrothers normal limits. Q1. Based on the history and clinical examination, what is the most likely diagnosis? a. Port-wine stain (PWS) b. Infantile hemangioma c. Nevus Anemicus d. Salmon patch Ans: a. Port-wine stain. Presence of nonprogressive, non- blanchable macule with unilateral distribution on the face suggests the possibility of PWS. Association of PWS with seizures may point toward Sturge-Weber syndrome (SWS). Q2. How do you diagnose PWS? Ans: Diagnosis of PWS: yy Diagnosis is made on clinical grounds. PWS, also called as nevus flammeus, presents as well to ill-defined pink to bright red macules at Fig. 6: Facial port-wine stain with slight hypertrophy of lower lip birth seen most commonly on the face, but can occur anywhere on the body including mucosae Jaypee(Fig. 6). They tend to darken with age and may yy Port-wine stains are mostly unilateral in develop small nodules leading to a cobble stoned distribution. Facial PWS may be associated with appearance. ocular and neurological abnormalities.
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Q3. How do you confirm your diagnosis? contralateral hemiparesis, EEG abnormalities and Ans: Though PWS is a clinical diagnosis, skin biopsy can be intracranial calcification on CT scan. done to rule out other disorders. Skin biopsy shows Q6. What are the changes that occur in PWS? proliferation and ectasia of thin and thick walled vessels Ans: Port-wine stain darkens with age, and secondary with lumina of varying diameter in the upper dermis. proliferative lesions like vascular blebs (pyogenic Q4. What are the other vascular abnormalities granuloma-like lesions) may develop on PWS. Rarely associated with PWS? basal cell carcinoma has been reported to occur in Ans: Various vascular abnormalities associated with PWS PWS. Soft tissue swelling and bony overgrowth may are hemangiomas, arteriovenous fistulae, nevus develop under a PWS. anemicus, pyogenic granuloma and tufted angiomas. Q7. What are the other syndromes associated with Q5. What are other associated features to look for in PWS? facial PWS? Ans: Syndromes associated with PWS are listed in Table 4. a. Ocular abnormalities Q8. How do you differentiate PWS from infantile b. Grand mal epilepsy hemangiomas? c. Subnormal intelligence Ans: Early PWS may resemble infantile hemangioma. d. Contralateral hemiparesis Congenital onset, static nature without rapid e. All of the above proliferation differentiates PWS from hemangioma. Ans: e. All of the above. Q9. What are the treatment modalities for PWS? Facial PWS in the distribution of trigeminal nerve can be associated with SWS. Ocular abnormalities a. Laser therapy are present in 40% of these patients and include b. Surgery ipsilateral glaucoma, buphthalmos, congestion, c. Radiation therapy strabismus and loss of vision. Glaucoma is seen d. Cryotherapy in two-thirds of cases in infancy. Grand mal type Ans: a. Laser therapy. Flashlamp-pumped tunable pulsed convulsions usually occur within first year of dye laser is the treatment of choice for PWS. It is done life. There may be other associated features like in several sessions 6–8 weeks apart. It is associated behavioral problems, subnormal intelligence, Brotherswith mild scarring and pigmentary changes.
Table 4: Syndromes associated with PWS Syndromes Clinical features Klippel-Trenaunay syndrome (KTS) PWS, venous and lymphatic malformation, bony and soft tissue hypertrophy Parkes-Weber syndrome PWS, venous varicosities, limb hypertrophy, arteriovenous fistulae Phakomatosis pigmentovascularis PWS, extensive nevus flammeus, pigmentary nevus, aberrant Mongolian spots, nevus anemicus, Beckers nevus, renal agenesis, features of SWS and KTS Proteus syndrome Overgrowth of multiple tissues, asymmetry, exostosis, disfigurement of skull, macrocephaly, partial gigantism of hands or feet, hemangioma, PWS, lipomas, lymphangioma, verrucous epidermal nevi Cobb syndrome Spinal cord angioma overlaid by segmental PWS or cavernous hemangioma Bannayan-Riley-Ruvalcaba syndrome Macrocephaly, mixed vascular malformation, subcutaneous and visceral lipomas, skeletal defects Beckwith-Wiedemann syndrome Facial PWS, macroglossia, omphalocele, visceral hyperplasia, hemihypertrophy Von Hippel-Lindau disease PWS, bilateral retinal angiomatosis, cerebellar or medullary hemangioma Rubenstein-Taybi syndrome Mental retardation, broad thumbs and great toes, high palate, crowded teeth, PWS Wyburn-Mason syndrome Ipsilateral PWS, unilateral retinal arteriovenous malformation, ipsilateral aneurismal arteriovenous malformation Coat’sJaypee disease Ipsilateral PWS, retinal telangiectasias
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