PATHOLOGY OF VASCULAR SKIN LESIONS - PART 1 pptx

C URRENT C LINICAL P ATHOLOGYIVAN DAMJANOV, MD SERIES EDITOR Pathology of Vascular Skin Lesions: Clinicopathologic Correlations, by Omar P.. Why a book on cutaneous vascular proliferatio

P ATHOLOGY OF V ASCULAR S KIN L ESIONS

C URRENT C LINICAL P ATHOLOGY

IVAN DAMJANOV, MD

SERIES EDITOR

Pathology of Vascular Skin Lesions: Clinicopathologic Correlations, by Omar

P Sangüeza, MD, and Luis Requena, MD, 2003

Practical Immunopathology of the Skin, by Bruce R Smoller, MD, 2002

L UIS R EQUENA , MD

Department of Dermatology Fundación Jiménez Díaz Universidad Autonoma Madrid, Spain

© 2003 Humana Press Inc.

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Due diligence has been taken by the publishers, editors, and authors of this book to assure the accuracy of the information published and to describe generally accepted practices The contributors herein have carefully checked to ensure that the drug selections and dosages set forth in this text are accurate and in accord with the standards accepted at the time of publication Notwithstanding, as new research, changes in government regulations, and knowledge from clinical expe- rience relating to drug therapy and drug reactions constantly occurs, the reader is advised to check the product information provided by the manufacturer of each drug for any change in dosages or for additional warnings and contraindications This is of utmost importance when the recommended drug herein is a new or infrequently used drug It is the responsibility

of the treating physician to determine dosages and treatment strategies for individual patients Further it is the bility of the health care provider to ascertain the Food and Drug Administration status of each drug or device used in their clinical practice The publisher, editors, and authors are not responsible for errors or omissions or for any consequences from the application of the information presented in this book and make no warranty, express or implied, with respect to the contents in this publication.

responsi-Cover design by Patricia F Cleary.

Cover illustration: Histopathologic features in plaque lesions of Kaposi’s sarcoma: The “promontory” sign evident

around preexisting capillaries (See Fig 9C in Chapter 9 and companion CD-ROM and discussion on pp 223–224.)

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Printed in the United States of America 10 9 8 7 6 5 4 3 2 1

Library of Congress Cataloging-in-Publication Data

Sangüeza, Omar P.

T o Catherine, Edith, and Charles,

in appreciation of her love and limitless patience.

Without her, nothing makes sense.

Why a book on cutaneous vascular proliferations? There are several compellingreasons to justify the existence of a book on this topic One of the most important is thatcutaneous vascular proliferations are exceedingly common and affect a large number ofindividuals of both sexes and within a wide age range They make up a broad spectrum

of lesions with morphological and biological variations, ranging from hamartomas tohighly malignant, aggressive neoplasms Although the diagnosis of some vascular lesions

is straightforward, many entities pose significant problems in diagnosis, classification,and treatment Within the past two decades there has been an increase in the number ofpatients affected with Kaposi’s sarcoma, related to the epidemic of the acquiredimmunodeficiency syndrome (AIDS) As a consequence, a number of variants andvascular lesions that simulate Kaposi’s sarcoma, both clinically and histopathologically,have been described In addition, other vascular entities not related to Kaposi’s sarcomahave been introduced in the literature All of these have added confusion to an alreadycomplicated field Since there are no recent textbooks on this subject, we felt an updatewas overdue

The aim of Pathology of Vascular Skin Lesions: Clinicopathologic Correlations is to

provide a comprehensive and in-depth review of all vascular proliferations involving theskin and subcutaneous tissue, including recently described entities Although our work

is primarily directed to pathologists, dermatologists, and dermatopathologists, its widescope will make it useful to pediatricians and plastic surgeons as well

Pathology of Vascular Skin Lesions: Clinicopathologic Correlations is divided into

three parts The first part covers classification and nomenclature of vascular neoplasms,

an area that is still controversial We propose a new classification with the hope that itwill bring more order into a chaotic arena We recognize that this classification may havesome pitfalls and limitations, but we also believe that it is the most logical way toapproach the study of vascular proliferations

In order to know what is abnormal, a student of the field should first know what isnormal, which is the reason for including a chapter on normal embryology, histology,and anatomy of the skin vasculature Another chapter is devoted to the use of specialtechniques for the study of vascular proliferations

In the second part, we include benign proliferations ranging from hamartomas andmalformations to benign neoplasms The final part of the book deals with malignantvascular proliferations, ranging from Kaposi’s sarcoma to angiosarcomas It includessome new entities, too

The whole of Pathology of Vascular Skin Lesions: Clinicopathologic Correlations

was conceived in terms of a clinicopathologic correlation The clinical and morphologic

vii

P REFACE

aspects of each entity are described in detail, including their differential diagnosis,prognosis, and therapy Each chapter is fully illustrated with both clinical andhistopathologic photographs, and we include color versions of all illustrations on theaccompanying CD-ROM Additionally, there is a complete and updated list of referencesfor each particular section We hope that you find this book interesting and useful.This book was sponsored in part by Pathologists Diagnostic Services, PLLC, inWinston-Salem, NC.

Omar P Sangüeza, MD

Luis Requena, MD

A CKNOWLEDGMENTS

Many colleagues contributed clinical pictures, histopathologic slides, or othermaterial for this review We are very grateful to the following clinicians andpathologists:

A Bernard Ackerman, MD (USA)Antonio Aguilar, MD (Spain)Adolfo Aliaga, MD (Spain)Isabel Febrer, MD (Spain)

M Alba Greco, MD (USA)Gerardo Jaqueti, MD (Spain)Esperanza Jordá, MD (Spain)Helmut Kerl, MD (Austria)Heinz Kutzner, MD (Germany)Pablo Lázaro, MD (Spain)Beatriz López, MD (Spain)José M Mascaró, MD (Spain)Thomas Mentzel, MD (Germany)

Special thanks to: Di Lu, MD (USA), who spent countless hours shooting photographs; Rita O Pichardo, MD (Venezuela), who helped to compile and organize allphotographic and written material, and to Steven Vogel, MD, who corrected the manu-script, offered support, and provided advice

micro-Figures 10 and 19 in Chapter 6, Figure 13 in Chapter 7, Figure 15 in Chapter 8, Figure

3 in Chapter 11, and Figure 35 in Chapter 8 have been previously published (J Am AcadDermatol 1977;37:523-49 J Am Acad Dermatol 1997;37:887-20 J Am Acad Dermatol1998;38:143-75) These figures are reproduced here with permission of Mosby Inc

Paula E North, MD (USA)Celia Requena, MD (Spain)Jorge L Sánchez, MD (Puerto Rico)Evaristo Sánchez Yus, MD (Spain)Pastor Sangüeza, MD (Bolivia)Andrés Sanz, MD (Spain)Jaime Tschen, MD (USA)Antonio Torrelo, MD (Spain)Sara O Vargas, MD (USA)Antonio Vélez, MD (Spain)Angel Vera, MD (Spain)Michel Wassef, MD (France)

ix

Preface vii Acknowledgments ix

Companion CD-ROM Inside Back Cover

1 Embryology, Anatomy, and Histology

of the Vasculature of the Skin 1

1 Embryologic Aspects 1

2 Anatomic and Histologic Aspects of the Dermis and Blood Vessels 2

2 Special Techniques for the Study of Vessels

and Vascular Proliferations 7

1 Immunohistochemical Stains 7

2 Molecular Techniques 10

3 Cytogenetic Studies 12

3 Classification of Cutaneous Vascular Proliferations 15

4 Cutaneous Vascular Hamartomas 19

1 Phakomatosis Pigmentovascularis 19

2 Eccrine Angiomatous Hamartoma 23

5 Cutaneous Vascular Malformations 27

6 Superficial Cutaneous Lymphatic Malformations 63

7 Cystic Lymphatic Malformations (Cystic Hygromas) 67

8 Lymphangiomatosis 70

6 Cutaneous Lesions Characterized by Dilation

of Preexisting Vessels 73

1 Spider Angioma (Nevus Araneus) 73

2 Capillary Aneurysm-Venous Lake 76

7 Cutaneous Vascular Hyperplasias 99

1 Angiolymphoid Hyperplasia with Eosinophilia 99

12 Giant Cell Angioblastoma 184

13 Spindle Cell Hemangioma (Formerly Spindle Cell

3 Endovascular Papillary Angioendothelioma (Dabska’s Tumor

or Papillary Intralymphatic Angioendothelioma) 241

4 Retiform Hemangioendothelioma 245

5 Composite Hemangioendothelioma 250

6 Cutaneous Angiosarcoma of the Face and Scalp of Elderly Patients

(Wilson Jones’ Angiosarcoma) 251

7 Cutaneous Angiosarcoma Associated with Lymphedema 258

10 Other Cutaneous Neoplasms

With a Significant Vascular Component 275

1 Multinucleate Cell Angiohistiocytoma 275

Chapter 1 / Vasculature of the Skin 1

1

1 Embryology, Anatomy, and Histology

of the Vasculature of the Skin

CONTENTS

EMBRYOLOGIC ASPECTS

ANATOMIC AND HISTOLOGIC ASPECTS OF THE DERMIS AND BLOOD VESSELS

The skin is a complex organ responsible for numerous physiologic and immunologic

functions It is conceptually the largest organ of the body (1) It weighs between 3 and

4 kg, constitutes 6% of body weight, and, on the average adult, covers an area of mately 2 m2 The functions of the skin are numerous and diverse Notably, it serves as abarrier that excludes harmful chemicals and pathogens while retaining water and endog-enous proteins The skin also modulates body temperature, acts as a sensory organ,protects against physical injury, is a component of the immune system, and has psycho-social and aesthetic importance It is composed of three principal layers: the epidermis,the dermis, and the subcutaneous tissue It also houses the adnexa, melanocytes, Langer-hans cells and Merkel cells

approxi-1 EMBRYOLOGIC ASPECTS

All components of the skin are derived embryologically from either the ectoderm orthe mesoderm The ectoderm gives origin to the epidermis and the epithelial dermalconstituents, whereas the mesenchymal components of the dermis are derived from themesoderm

The earliest evidence of skin is seen at the end of the first month of embryonic life, at

which time a single layer of cuboidal epithelium encases the embryo (2) By the fourth

to sixth weeks of gestation, this epithelium has evolved into a two-cell layered structure.The outer layer, or periderm, is composed of glycogen-laden cells, superimposed upon

cuboidal cells that form the inner or basal layer (2) The periderm is in immediate contact

with the amniotic fluid, into which these cells are gradually shed to the point of totaldisappearance by the 21st week of estimated gestational age (EGA) An intermediatelayer develops between the periderm and basal layer by the 11th week At this point, theinner layer evolves into the stratum germinativum, which continues to proliferate and toserve as the source of epidermal cells throughout life By the 21st week of EGA, theintermediate layer has differentiated into the stratum spinosum, granulosum, and cor-neum The first semblance of cornification is seen after the fifth gestational month

2 Sangüeza and Requena / Pathology of Vascular Skin Lesions

Thereafter, there is increased production of keratohyalin granules, and the epidermalcells near the surface lose their nuclei Complete cornification is normally accomplished

by the sixth month of EGA

The dermo-epidermal junction develops during the first trimester, and all the elements

of this layer are recognizable thereafter (3) The components of the basal layer are

pro-duced by the basal cells of the epidermis During the first 3 months of intrauterine life,cells migrate from the neural crest to the epidermis, where they become melanoblasts andpresumptively also Merkel cells Merkel discs are recognizable by the 7th month, whereas

melanocytes can be identified with special stains by the 10th or 11th week (4)

Langer-hans cells are derived from the bone marrow to serve as immunomodulators At entry intothe epidermis by the seventh week, they differ from mature Langerhans cells since they

express different antigens (5) Human leukocyte antigen-DR, as well as the CD1 antigen,

normal constituents of mature Langerhans cells, can be recognized by the 12th week of

EGA, whereas Birbeck granules can be identified ultrastructurally 2 weeks earlier (3).

Folliculo-sebaceous-apocrine units appear around the ninth week, initially in the headand neck, notably in areas of the future eyebrows, eyelids, upper lip, and chin Theydevelop in a cephalocaudal direction, and by the fourth month hair follicles are alsoevident in the abdominal skin and elsewhere Most of the hair follicles are present by the

fifth month, and probably no new hair follicle formation takes place after birth (2).

Sebaceous glands remain appended to hair follicles in extrauterine life, but most apocrineglands involute shortly after birth and remain present only in select cutaneous areas,notably the axillae, genital area, and mammary areola

Eccrine glands appear on the palms and soles by the 12th week of EGA They originate

as small proliferations of the basal layer, as protusions into the dermis and epidermis Inthe dermis they form unbranched, highly coiled glands whereas in the epidermis theyform the acrosyringium Centrally positioned cells in these proliferations degenerate tocreate the lumen of the gland; the peripheral cells differentiate into an inner layer ofsecretory cells and an outer layer of myoepithelial cells

During the first 5–7 weeks of intrauterine life, the dermis is mostly cellular During thisearly interval, there is no sharp demarcation between the dermis and the subcutaneous fat,and there are no recognizable adnexal structures within the connective tissue Betweenthe 8th and 9th weeks, the amount of collagen increases markedly in the extracellularmatrix, so consequently by the 10th–12th weeks the cellular dermis has been transformedinto a predominantly fibrous dermis During this interval, the deep boundary of thedermis is defined by a plexus of blood vessels and nerves that lie in a plane betweenthe dermis and subcutaneous fat Once the dermis has become predominantly fibrous, as

it does by approximately the 13th week of EGA, vessels and nerves are recognizable, atall levels of the dermis, unsheathed by connective tissue Definite papillary and reticular

dermis is distinguishable by the 14th week (6) Nerve endings are recognizable during

the fourth gestational week and continue to increase in number thereafter until the seventh