Diseases of the Hair and the Scalp. Current Problems Dermatology. Vol. 12. Series Editor J. W. H. Mali, Nijmegen KARGER

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Diseases of the Hair and the Scalp Current Problems Dermatology Vol. 12 Series Editor J. W. H. Mali, Nijmegen KARGER S. Karger Basel Mnchen. Paris London New York Tokyo Sydney Diseases of the Hair and the Scalp L. Bartosou, V. lorda, z. Stva 83 figures, 1 table, 1984 KARGER S. Karger Basel Mnchen. Paris London New York Tokyo Sydney Current Problems in Dermatology National Library of Medicine, Cataloging in Publication Bartosov, L. (Ludmila) Diseases of the hair and the scalp /L. Bartosova, V. Jorda, Z. Stava. - Basel; New York: S. Karger, 1984. (Current problems in dermatology; v. 12). Includes index. 1. Hair Diseases 2. Scalp Dermatoses I. Jorda, V. (Vladimir) II. Stva, Zdenek III. Title IV. Series ISBN 3805537832 WI CU804L v. 12 [WR 450 B293d]

All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechamcal, includmg photo' copying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Copyright 1984 by Avicenum, Czechoslovak MedIcal Press, Prague Printed in the CSSR S. Karger AG, P.O. Box, CH4009 Basel (Switzerland) has the sale distribution rights for all countries with the exception of the socialist countries. ISBN 3-8055-3783-2 Contents Preface BIOLOGY OF HAIR GROWTH (L. Bartosova) 1. Macroscopic Types of Hair...1 2. Development of Hair Follicles...6 3. Anatomy of the Follicle and the Hair...16 3.1. Microstructure of the Follicle and the Hair...16 3.2. Ultrastructure and Differentiation of the Bulb Cells..24 3.3. Keratinization and the Composition of Hair Keratin...30 3.4. Hair Pigmentation.33 4. Cyclic Hair Growth (Cyclic Activity of the Follicle)...36 4.1. Phases of Cyclic Hair Changes.36 4.2. Mechanism of Hair Changes.39 4.3. Melanocyte Changes during the Hair Growth Cycle..42 4.4. Interrelationship of the Follicular and the Skin Cycle in Laboratory...43 Animals...43 5. Regulation of Hair Growth. 45 5.1. The Relationship of Nerves and Blood Vessels to Hair Growth. 45 5.2. Hormonal Factors... 47 5.2.1. Androgen Metabolism in Cells... 50 5.3. The Role of Nutrition in Hair Growth 51 6. Induction and Inhibition of Hair Growth... 54 7. Follicle Neogenesis...57 CLINICAL TRICHOLOGY (L. Bartosovta, Z. Stava, V. Jorda}... 59 1. Introduction to Clinical Trichology fl. Bartosovti}... 59

2. Alopecia... 62 2.1. Androgenic Alopecia in Males (Synonyms: Common Baldness, Male-pattern Alopecia) 62 Contents VI 2.2. Alopecia Areata (Synonyms: Area Celsi, Pelade) 68 2.3. Diffuse Alopecia and Hypotrichosis (Z. Stava) 79 2.3.1. Telogen Effluvium... 79 2.3.2. Diffuse Alopecia of Endocrine Origin... 80 2.3.3. Alopecia of Nutritional and Metabolic Origin... 83 2.3.4. Diffuse Alopecia due to Drugs and Chemicals... 84 2.3.5. Diffuse Alopecia in Internal Diseases and Disorders of the Central Nervous System... 87 2.3.6. Chronic Diffuse Alopecia of Unknown Origin... 88 2.3.7. Traumatic Alopecia... 90 2.4. Cicatricial Alopecia...93 2.4.1. Traumatic Cicatricial Alopecia...94 2.4.'2. Genetically Determined Cicatricial Alopecia...95 2.4.3. Clinical Syndromes of Unknown Origin... 97 2.5. Inborn, Often Hereditary Alopecia and Hypotrichosis.. 104 2.5.1. Circumscribed Alopecia....104 2.5.2. Inborn Diffuse Alopecia and Hypotrichosis... 105 3. Excessive Hair Growth and Changes in Distribution; Hypertrichosis and Hirsutism...113 3.1. Hypertrichosis... 113 3.1.1. Hypertrichosis (universalis) lanuginosa...113 3.1.2. Hypertrichosis universalis congenita...115 3.1.3. Symptomatic Hypertrichosis....116 3.1.4. Circumscribed Hypertrichosis...121 3.2. Hirsutism...123 3.'2.1. Idiopathic Hirsutism... 126 3.2.2. Adrenal Syndromes... 127 3.2.3. Ovarian Syndromes...128 3.2.4. Iatrogenic Hirsutism... 130 3.2.5. Differential Diagnosis of Hirsutism... 131 3.2.6. Biochemical Investigations...133 3.2.7. Treatment... 134 4. Abnormalities of Hair Structure... 135 4.1. Variations in Hair Shaft Structure...135 4.2. Hereditary and Congenital Anomalies of the Hair Shaft....136 4.2.1. Monilethrix... 136 4.2.2. Pseudomonilethrix...141

4.2.3. Pili torti....142 4.2.4. Menkes' Kinky Hair Syndrome (Synonyms: Trichopoliodystrophy, Stubby Hair Disease, Steely Hair Disease)... 144 4.2.5. Bj~rnstad's Syndrome and Crandall's Syndrome... 146 4.2.6. Woolly Hair... 146 4.2.7. The "CHAND" Syndrome...148 4.2.8. Curly Hair in Other Hereditary Syndromes... 148 4.2.9. Bayonet Hairs... 148 4.2.10. Trichorrhexis invaginata (Bamboo Hairs)...149 4.2.11. Trichomalacia....150 Contents VII 4.2.12. Trichoschisis... 150 4.2.13. "Uncombable" Hair...150 4.2.14. Uncharacterized Anomalies...151 4.2.15. Trichostasis spinulosa (Synonym: Pinselhaare)...151 4.3. Exogenous Hair Damage...152 4.3.1. Trichorrhexis nodosa..152 4.3.2. Trichonodosis...155 4.3.3. TrichoptiloSis...156 4.3.4. Pili bifurcati...157 4.3.5. Trichothiodystrophy... 157 5. Hair Pigmentation Defects (V. Jorda)... 157 5.1. Introduction: Melanogenesis - Summary, Pathogenesis of Hair Anomalies, Individual and Racial Variation of Hair Colour, Pigment in Redheads (Rutilism)...157 5.2. Canities (Greying of the Hair) (Synonyms: Leucotrlchia, Achromo trichia)...163 5.2.1. Premature Canities...166 5.2.2. Poliosis... 168 5.3. Heterochromia pilorum...173 5.4. Pili anm.dati (Synonyms: Leucotrichia annularis, Ringed Hair)...174 5.5. Hair Colour Changes Associated with Growth Disturbances....176 5.6. Nutritional, Metabolic and Chemical Factors Influencing Hair Colour...176 5.7. Accidental and Artificial Discoloration of the Hair (Including Dyeing, Toning and Bleaching)...179 5.7.1. Cosmetic Hair Dyes....180 5.7.2. Hair Colorants...183 5.7.3. Bleaches...184 DISEASES OF HAIRY SKIN (V. Jorda) 185

1. Infectious and Other Conditions..186 1.1. Fungus Infections....186 1.1.1. Deep Trichomycoses...186 1.1.2. Microsporosis... 190 1.1.3. Favus...192 1.2. Pyogenic Infections... 194 1.2.1. Folliculitis...194 1.2.2. Acne necrotica (Synonyms: Acne necroticans, Acnee varioliformis)...196 1.2.3. Furuncle and Carbuncle... 197 1.3. Bacterial Infections...198 1.3.1. Tuberculosis of the Skin...198 1.3.2. Syphilis....198 1.3.3. Leprosy....200 1.4. Virus Infections...200 1.4.1. Herpes zoster...200 1.4.2. Varicella (Chickenpox)...201 Contents 1.4.3. Variola (Smallpox)...201 1.5. Lichen planus of the Scalp...202 1.6. Lupus erythematosus...204 1.7. Pemphigus...205 1.7.1. Pemphigus vulgaris...207 1.7.2. Benign Mucosal Pemphigoid...207 1.8. Scleroderma...208 1.9. Sarcoidosis (Synonym: Besnier-Boeck-Schaumann Disease)...210 1.10. Cutis verticis gyrata...210 2. Naevi and Cysts...211 3. Benign and Malignant Tumours...212 4. Keratinization Disorders of the Skin...216 4.1. Pityriasis (simplex) capitis (Synonyms: Pityriasis capillitii, rhoea slcca, Pityriasis sicca)...216 4.2. Pityriasis steatoides (Synonym: Seborrhoea oleosa ]..218 4.3. Seborrheic Dermatitis (Synonym: Eczema seborrhoicum)...219 4.4. Pityriasis amiantacea (Synonyms: Tinea (pseudotinea) amiantacea,tinea asbestlna)...220 4.5. Psoriasis of the Scalp. 221 4.6. Irritant and Allergic Contact Dermatitis. 223 LABORATORY AND EXPERIMENTAL TRICHOLOGY (L. Bartosova, V. jorda)... 224

1. The Trichogram - Diagnostic Epilation...224 2. Methods of Examination of the Hair Surface...229 3. Measurement of Mechanical Hair Properties...231 4. Classification and Measurement of Hair Colour...223 5. Measurement of Hair Growth...233 6. Hair Tissue Cultures...234 References...239 Subject Index...243 Preface From the aesthetic and the medical aspect, hair is a striking component of man's outward appearance, while biologically it is an attractive material for research. It is thus not surprising that "hair research" has a glorious past and present which have made trichology a field with a wide range of interests, from the generally practical to the highly specialized. The former includes unresolved clinical questions related to the problematical treatment of what ouen seem to be banal trichological lesions. Experimental medicine and theoretical (especially biological) branches attempt to resolve basic problems of clinical trichology. The function of the hair follicle is relatively easy to evaluate, but the biological nature of what appears, at first glance, to be a simple activity - the production of hair - and a readily identifiable function is actually still comparatively obscure. Knowledge of macroscopic differences in the hair of different races and ethnic groups is probably the most complete. In the foreground of these essentially anthropological studies is the description of the distribution, quantity, pigmentation and type of the hair in correlation to sex and physiological development. The findings furnish a given norm which must be taken into account in the clinical evaluation of trichological changes, since there is a series of genetically determined variations which occur only at given ages and are merely individual, and not pathological, anomalies. Preface...x Further groups of trichological problems, e. g. follicular

cell metabolism, keratinization, the physical properties, structure and ultrastructure of the hair shaft, pigmentogenesis, pigment biochemistry and ultrastructure, the association of pilary function with genetic, endocrine, metabolic and other factors and utilization of the findings in hair growth pathology, are closely associated with general biological questions. Linked to these basic studies is experimental trichology, which investigates the effects of various factors on the morphology and function of the follicle. The results of some of the situations induced in laboratory animals are not absolutely applicable to hair growth in man, however, and in clinical trichology classic examination methods must therefore be used. Macroscopic differences, e. g. in the thickness and distribution of the hair, or changes in the structure and pigmentation of the hair shaft, can be due to complex internal causes of metabolic, endocrine and gentic origin, etc., or to some later pathological process. A general examination of the patient is therefore necessary, supplemented, in some cases, by histological tests, a trichogram and, in obscure cases, by a genealogical investigation. Rathmann [33] called the pilary system a complete microcosmic structure in which we can find birth, development, aging and death, activity and rest, the development and disappearance of pigment, greasiness and dryness, infection and sterilization, hypertrophy and atrophy, benign tumours and malignant ones. It is the perfect entirety of the fundamental processes which take place in the follicle under both physiological and pathological conditions and of which we practically still possess only incomplete, fragmentary knowledge. On the other hand clinical trichology, including knowledge of scalp diseases, has made great progress during the last decades, mainly owing to the collaboration of various branches of medicine. This applies to the diagnosis and classification of many new entities, deeper knowledge of their pathogenesis, research on their genetic pattern and/or external conditioning, and last but not least, their effective treatment or prevention. Therefore, more than two thirds of our monograph deal with these recent and practical matters.