Diseases of the Hair and Scalp dad Peet eer | ey Pere | rider Pr |Diseases of the Hair and Scalp EDITED BY RODNEY DAWBER MA, MB, ChB, FRCP Consultant Dermatologist, Department of Dermatology, Churchill Hospital, Oxford THIRD EDITION b Blackwell Science© 1982, 1991, 1997 by Blackwell Science Ltd Editorial Offices: Osney Mead, Oxford OX2 oEL 25 John Street, London WC1N 2BL 23 Ainslie Place, Edinburgh EH3 6AJ 350 Main Street, Malden MA 02148 5018, USA 54 University Street, Carlton Victoria 3053, Australia Other Editorial Offices: Blackwell Wissenschafts-Verlag GmbH Kurfiirstendamm 57 10707 Berlin, Germany Blackwell Science KK MG Kodenmacho Building 7-10 Kodenmacho Nihombashi Chuo-ku, Tokyo 104, Japan All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the copyright owner. First published 1982 Second edition 1991 Third edition 1997 Set by Excel Typesetters Co., Hong Kong Printed and bound in Italy , by G. Canale & C, SpA, Turin The Blackwell Science logo is a trade mark of Blackwell Science Ltd, registered at the United Kingdom Trade Marks Registry DISTRIBUTORS Marston Book Services Ltd PO Box 269 Abingdon Oxon OX14 4YN (Orders: Tel: 01235 465500 Fax: 01235 465555) USA Blackwell Science, Inc. Commerce Place 350 Main Street Malden, MA 02148 5018 (Orders: Tel: 800 759 6102 617.388 8250 Fax: 617 388 8255) Canada Copp Clark Professional 200 Adelaide St West, 3rd Floor Toronto, Ontario MsH 1W7 (Orders: Tel: 416 597-1616 800 815-9417 Fax: 416 597-1617) Australia Blackwell Science Pty Ltd 54 University Street Carlton, Victoria 3053 (Orders: Tel: 3 9347 0300 Fax: 3 9347 5001) A catalogue record for this title is available from the British Library ISBN 0-865 42-866-2 Library of Congress Cataloging-in-publication Data Diseases of the hair and scalp/ edited by Rodney Dawber.—3rd ed. . cm. Includes bibliographical references and index. ISBN 0-865 42-866-2 1. Hair—Diseases. 2. Scalp— Diseases. I. Dawber, R.P.R. © (Rodney P. R.) [DNLM: 1. Hair Diseases. 2. Scalp Dermatoses. WR 450 D611 1997] RL151.D56 1997 616.5/46—dce21 DNLM/DLC for Library of Congress 96-45303 CIPContents List of contributors, vi Preface to the third edition, vii Preface to the first edition, viii The physiology and embryology of hair growth, 1 A.G. Messenger & R.P.R. Dawber Hair follicle structure, keratinization and the physical properties of hair, 23 R.P.R. Dawber & A.G. Messenger The hair in infancy and childhood, 51 ].H. Barth Hair patterns: hirsuties and androgenetic alopecia, 67 N.B. Simpson @& J.H. Barth Diffuse alopecia: endocrine, metabolic and chemical influences on the follicular cycle, 123 R.P.R. Dawber, N.B. Simpson & J.H. Barth Hereditary and congenital alopecia and hypotrichosis, 151 R. Sinclair & D. de Berker Defects of the hair shaft, 239 D. de Berker & R. Sinclair Hypertrichosis, 299 J... Barth Io II Iz 13 14 TS 16 17 18 19 Traumatic alopecia, 328 R.P.R. Dawber & C.L. Gummer Alopecia areata, 338 A.G. Messenger & N.B. Simpson Cicatrical alopecia, 370 R.P.R. Dawber & D.A. Fenton The colour of the hair, 397 R.P.R. Dawber @& C.L. Gummer Infections and infestations, 418 R.P.R. Dawber & D.A. Fenton Psychological factors and disorders of the hair, 461 R.P.R. Dawber Hair cosmetics, 466 C.L. Gummer & R.P.R. Dawber Hair and scalp in systemic diseases, 483 R.P.R. Dawber & N.B. Simpson Diseases of the scalp and skin diseases involving the scalp, 494 R.P.R. Dawber & D. de Berker Naevi, tumours and cysts of the scalp, 528 R.P.R. Dawber Measurement of hair growth and investigation of hair disease, 564 J.H. Barth @ A.G. Messenger Index, 581List of contributors J.H. BARTH Department of Chemical Pathology and Immunology, Institute of Pathology, The General Infirmary at Leeds, Leeds LSt 3EX D. de BERKER Department of Dermatology, Bristol Royal Infirmary, Bristol BS2 8HW R.P.R. DAWBER Department of Dermatology, Churchill Hospital, Oxford OX3 7L] D.A. FENTON Department of Dermatology, St Jobn’s Institute of Dermatology, St Thomas’ Hospital, London SE1 7EH vi C.L. GUMMER Procter and Gamble (H & BC) Europe, Egham TW20 9NW A.G. MESSENGER Department of Dermatology, Royal Hallamshire Hospital, Sheffield Sto 2JF N.B. SIMPSON Department of Dermatology, Royal Victoria Infirmary, Newcastle upon Tyne NExz 4LP R. SINCLAIR Department of Dermatology, St Vincent's Hospital, Melbourne, AustraliaPreface to the third edition Since the last edition our knowledge of many aspects of hair biology, pathology and therapeutics has expanded almost exponentially! Because of this several sections of this edition have been almost totally reorganized. Rod Sinclair, David de Berker and Andrew Messenger have joined the writing team, adding much to the areas of hair structure in health and disease, hair growth control mechanisms and alopecia areata — fields in which they are world authorities. However, as none of the writers are hair surgeons it was decided once more not to expand the book to accommodate this topic as it is covered expertly and comprehensively in every major textbook of dermatological surgery. - Those in dermatology will be aware that since the last edition was in preparation, my co-editor, Dr Arthur Rook, has died. His immense scholarship during the writing of the first edition was massively educational for me and those who knew of his editor- ial, academic and historical skills will recognize that he lives on in this edition, mainly in the detailed clinical historical areas. However, this book is not meant solely as a prag- matic guide for practising dermatologists; it is hoped that our collective experience will also make this volume of use to hair scientists, paediatricians, tricho- logists and cosmeticians and in particular geneticists. These are exciting times for molecular genetics and this is reflected in Chapters 6 and 7 on hereditary follicular and hair shaft abnormalities. There is also much more information on genetic mechanisms, but as yet no treatments! We also hope that now that all the clinical figures are in colour it will prove more useful as a diag- nostic manual than previous editions. We should again point out that this edition is not meant to be ‘everything on hair’; it thus reflects our particular interests to some degree. Rodney Dawber vilPreface to the first edition With a few notable exceptions most physicians, includ- ing most dermatologists, took little interest in disorders of the hair, apart from ringworm and alopecia areata and certain rare hereditary disorders of the hair shaft, until the development of scientific endocrinology in the present century provided some understanding of the mechanisms underlying certain common disturbances of hair growth. Recent research has greatly increased our knowledge of the complex endocrine influences on the hair, and has also established that a wide range of other metabolic and nutritional disturbances, and some psychiatric states, may first be clinically manifest as, or be accompanied by, changes in the density, pattern, colour or texture of the hair. Apart from those abnormalities of the hair which result from direct external infection, or from chemical or physical trauma, almost all are caused by or are related to sys- temic processes. The patient who complains of loss of hair, or of the growth of hair which she considers abnormal or excessive, is presenting her physician with a symptom which is as worthy of careful study and investigation as is abdominal pain or cough or any other symptom. viii Research by anthropologists and zoologists has thrown much light on the origin and significance of certain common changes in hair pattern, which have in the past wrongly been regarded as abnormal. They have also made it clear that there is no scientific justifi- cation for the study of the scalp hair in isolation from the reduced but far from vestigial hair coat in other regions of the body. This book attempts to present a practical clinical account of the hair and its disorders. It is hoped that it will be of value not only to the dermatologist, but also to other physicians who wish to understand the signifi- cance of changes in their patients’ hair. The compara- tive physiology of hair growth is described because it throws light on clinical situations in man. The history of each disorder is discussed briefly, where it explains international inconsistencies in nomenclature, and at greater length where it explains how discredited ‘scien- tific’ theories of the past have taken a prominent and sometimes a misleading place in contemporary folklore. Diseases of the scalp are included because they are so often associated with some disturbance of hair growth. Arthur Rook Rodney DawberChapter 1 / The physiology and embryology of hair growth A.G. MESSENGER & R.P.R. DAWBER Introduction, 1 Embryology, 2 Types of hair, 5 The hair cycle, 5 Introduction Hair is a characteristic feature of mammals. The factors which control hair growth and replacement in some mammals other than man have been extensively studied for a variety of motives. Economic pressures have certainly provided a stimulus for research into hair growth in the sheep and other species which directly or indirectly serve man’s needs. In addition, numerous experimental investigations have been carried out on the common laboratory animals. These studies in other mammals are of great importance in clinical practice for they throw light on the origin and significance of the complex mechanisms by which the growth and replacement of human hair are regulated. Although a striking feature of hair in man is its relative sparcity, where it is present it is often long and plentiful and by no means vestigial (Goodhart 1960). Man has largely lost the general covering of body hair which protects the skin of other primates. Ashley Montagu (1964) suggests that this reduction in body hair may have followed the hunting way of life which necessi- tated the development of a mechanism for the rapid loss of body heat. The eccrine glands were evolved and selection pressures then favoured the partial loss of the covering of hair which impaired their function. The head hair and beard are adornments directly concerned with sexual display (Patzer 1985); many authors writing in the fields of ethnology, anthropology, soci- ology and psychology have suggested more profound Systemic control of the hair cycle, 8 Hormonal control factors of human hair growth, 9 Intrinsic control of the hair cycle, 12 The dynamics of hair growth, 19 The trichogram, 20 The rate of hair growth, 20 functions for adult body hair — even mystical and magical significance (Leach 1958). Pubic hair is in general much better developed in man than in other species and axillary hair is an almost exclusive huaman characteristic. It is probable that the hair in both sites is concerned with the wider dissemination of the odour of the apocrine glands, which become functional at the age at which this hair develops. The general covering of body hair in many mammals has an important function in conserving heat, and in some the colour or pattern of colours serves as camouflage. In mammals living in geographical regions in which there are marked sea- sonal changes in temperature, a heavy coat which made survival possible could well be a handicap in warmer weather. Moulting probably evolved under such condi- tions to allow the necessary seasonal adjustment of the weight (and in some species also of the colour) of the coat. REFERENCES Goodhart, C.B. (1960) The evolutionary signification of human hair patterns and skin colouring. Advances in Science, 17, 53. Leach, E.R. (1958) Magical hair. Anthropological Institute of Great Britain and Ireland, 88, 147. Montagu, A. (1964) Natural selection and man’s relative hair- lessness. Journal of the American Academy of Dermato- logy, 187, 357. Patzer, G.L. (1985) The Physical Attractiveness Phenomena. Plenum Publications, New York.Chapter 1 (i) (b) Fig. r.x Early stages of hair follicle development in human foetal skin (a) from Olsen, E.A. (1994) Disorders of Hair Growth, McGraw-Hill Inc, New York, USA. Part (b) shows the formation of the epidermal hair peg; (i) oblique growth of the peg; (ii) the tip of the peg has become concave and encloses the dermal papilla. The upper bulge represents the Embryology (References, p. 4) A knowledge of the embryology of hair is valuable for the dermatologist, not only because it may eventually lead to an understanding of many of the genetic disor- ders of hair growth, but also because the sequence of events by which the hair follicle is formed in fetal life is partly recapitulated in each adult cycle of follicular activity suggesting that similar regulatory mechanisms are operating (Fig. 1.1a and b). The early work on the embryology of hair was carried out in Germany. Alber von Kolliker (1817-1905), Swiss by birth, became Pro- fessor of Anatomy in Wurzburg in 1847, and was a pioneer in the application of the cell theory in compar- ative anatomy and embryology. In 1850 he published an important article on the embryology of the skin in the journal of which he was co-founder (Kolliker 1850). P.G. Unna (1850-1929) of Hamburg was the first dermatologist to give serious attention to this subject (Unna 1876). The few English-language texts (iii) (ii) Developing sebaceous gland __— — Epidermis Hair peg Developing fs ae arrector pili f Sa muscle - Dermal papilla (iv) Sebaceous gland Hair 7 Arrector Internal '.” pili muscle root sheath Hair cone —~ Dermal papiila future sebaceous gland; the lower bulge, the site of attachment of the arrector muscles. In (iii) the sebaceous gland has formed. The cone of the internal root sheath is evident; (iv) the tip of the hair emerges from the protection of the internal root sheath. that did not ignore completely the embryology of the hair quoted Stohr’s Histology, which also appeared in English translation in 1896. The German work became still more widely known with the publication in 1910 of the Manual of Human Embryology, edited by Keith and Moll, to which F. Pinkus (1910) contributed an important chapter. The scientific approach in the United States to disease of the hair, based on studies of the embryology and physiology of the hair follicle, was given great impetus by Martin Engman, Professor of Dermatology, Washington University, St Louis, who had worked for a year in Unna’s clinic. Engman initi- ated a long-term research programme, in which C.H. Danforth, Mildred Trotter, L.D. Cady and others took part. The publications of this group (Danforth 1925) laid the foundations for much subsequent work on the hair. This account of the embryology of the human hair follicle is based largely on the writings of Pinkus (1958), Sengel (1976) and Spearman (1977). The first signs of hair follicle development occurbetween 9 and 12 weeks of gestational age in the eyebrow region and on the upper lip and the chin (Fig. 1.1a). These are the sites at which vibrissae are present in mammals other than man. This pre-germ stage of development is seen as a crowding of cells in the basal layer of the fetal epidermis. Electron microscopic studies (Breathnach & Smith 1968) showed that the initial crowding of epithelial cells in the pre-germ stage is not at first associated with other significant changes in the cells concerned. More or less simultaneously, mesenchymal cells begin to aggregate directly beneath the developing epithelial component. Cells in the basal layer elongate to form the hair germ which, as it enlarges, becomes asymmetrical and grows obliquely downwards. This solid column of cells, now known as the hair peg, the broad tip of which becomes slightly concave, carries before it the aggregation of mesenchy- mal cells which will form the dermal papilla and dermal sheath in the developed follicle. The cellular population of the dermal papilla is established at an early stage in development and thereafter is thought to remain stable throughout successive adult hair cycles (Wessels & Roessner 1965; Pierard & de la Brassinne 1975). As the follicle elongates the lower end becomes bulbous (the bulbous hair peg), and the cavity at the tip deepens to enclose the dermal papilla. Hair follicle development proceeds in a cephalocaudal direction and is completed by about 22 weeks. During the hair peg stage two swellings appear at the posterior side of the follicle. The upper swelling is the precursor of the sebaceous gland; the lower swelling (bulge or ‘Wulst’) is the impending site of attachment of the arrector muscle. In many follicles a third swelling appears above the sebaceous gland bud. In some areas of the skin, notably in the axillae, groins, genital skin, areolae and face, this third swelling will form the apocrine gland. Elsewhere it involutes. Between the epithelial cells melanocytes can be seen, and at first these are scattered throughout the lower part of the bulb and in the epithe- lial column. As hair follicle development proceeds, functionally active melanocytes are found only in that portion of the bulbous hair germ which will form the hair bulb matrix. The mesenchymal cells surrounding the bulb begin to form the dermal sheath. Above the matrix a cone of cells differentiates from The physiology/embryology of hair growth the matrix; these will form the hair. A second concen- tric cone surrounding the first is the future inner root sheath. The outer of the three components of the inner root sheath differentiates first as Henle’s layer; inside this Huxley’s layer and then the cuticle of the inner root sheath, the overlapping tile-like cells of which project downwards towards the base of the follicle. The differ- entiation of Henle’s and Huxley’s layers reaches an advanced stage before presumptive cuticular cells can be detected (Robins & Breathnach 1970). The inner cone gives rise to the cortex and cuticle of the hair shaft; there is no medulla in fetal hair. The cone of the inner root sheath extends upwards over the developing hair. However, upward growth of the hair and inner root sheath probably do not occur until the follicle has reached its full size. Lumen formation below the level of the sebaceous gland bud occurs by a process of cellu- lar necrosis. Above this level, lumen formation in the future infundibulum occurs by a combination of necro- sis and keratinization. The first hair coat of fine lanugo hair is shed in utero at about one month before birth at full term (Kligman 1961; Pecoraro & Astore 1990). The second coat of shorter lanugo, in all areas except the scalp where the hair may be both longer and of larger calibre, is shed during the first three or four months of life, almost imperceptibly, or as a wave terminating in almost complete alopecia. These first and second coats are synchronized in growth and in the sequence of shedding. The more or less unsyn- chronized mosaic pattern of hair growth then becomes established. Tissue recombination experiments have shown that development of skin appendages depends on a series of interactive messages passing between the dermis and the overlying epidermis (Kollar 1970; Sengel 1976; Hardy 1992). The initial message is derived from the dermis and instructs the epidermis ‘to form an appendage. This signal is common to all classes of ver- tebrate; thus, mouse dermis is able to instruct chick epi- dermis to initiate development of a feather follicle and chick dermis will initiate hair follicle development in mouse epidermis. This is followed by a less well- defined but class-specific signal from the epidermis which instructs the dermis to form a dermal papilla. Finally, a second dermal message stimulates the epider-