Chapter 01
Histology of the normal skin 1
The skin as an organ for barrier function 1
Epidermis 1
Desmosome 4
The type of cells in the epidermis 5
Senile skin 8
Mucosal epithelium 9
Dermal-epidermal junction 10
Dermis 11
Cutaneous vasculatures 11
Cutaneous nerves 14
Normal hairs 15
Hair cycle 16
Structure of normal hair follicle 16
Nail unit 22
Exocrine glands of the skin 24
Sebaceous glands 24
Eccrine glands 25
Apocrine glands 27
Subcutaneous fat 28
Histochemistry 28
Immunohistochemistry 28

Chapter 02
Genodermatoses 33
Ichthyosis 33
Ichthyosis vulgaris 35
X-linked Ichthyosis 36
Arthrogryposis-renal dysfunction-cholestasis (ARC)
syndrome 37
Keratinopathic ichthyosis 39
Epidermolytic ichthyosis 39
Ichthyosis bullosa of Siemens 41
Autosomal recessive congenital ichthyosis 42
Congenital ichthyosiform erythroderma 43
Lamellar ichthyosis 45
Harlequin ichthyosis 46
Ichthyosis follicularis alopecia photophobia syndrome 47
Peeling skin syndrome 49
Acral peeling skin syndrome 50
Palmoplantar keratoderma (PPK) 51
Diffuse nonepidermolytic palmoplantar keratoderma 52
Nagashima-type palmoplantar keratoderma 54
Focal, striate, and punctate palmoplantar keratoderma 55
Pachyonychia congenita 57
Porokeratosis 58
Xeroderma pigmentosum 60
Epidermolysis bullosa 62
Epidermolysis bullosa simplex (EBS) 63
Junctional epidermolysis bullosa (JEB) 65
Dystrophic epidermolysis bullosa (DEB) 66
Kindler syndrome 68
Hailey-Hailey disease 69
Darier's disease 70
Pseudoxanthoma elasticum 72
Connective tissue nevus 73
Ehlers-Danlos Syndrome 75
Cutis laxa 76
Tuberous sclerosis 78
Pachydermoperiostosis 80
Focal dermal hypoplasia 81
Anhidrotic (hypohidrotic) ectodermal dysplasia 83
Olmsted syndrome 84
Mastocytosis 86
Urticaria pigmentosa 87
Cutaneous mastocytoma 88
Diffuse cutaneous mastocytosis 89
Telangiectasia macularis eruptiva perstans 91
Incontinentia pigmenti 92

Chapter 03
Eczema and dermatitis 94
Introduction 94
Eczema (spongiotic dermatitis) 94
Contact dermatitis 96
Systemic contact dermatitis 98
Cutaneous allergic reaction to Vitamin K1 injection 99
Phytophotodermatitis 100
Hand eczema 101
Chronic actinic dermatitis 103
Nipple eczema 104
Xerotic eczema 105
Nummular eczema 105
Lichen simplex chronicus 107
Atopic dermatitis 108
Atopic eruption of pregnancy 110
Seborrheic dermatitis 113
Stasis dermatitis 114
Senile gluteal dermatosis 115

Chapter 04
Urticaria, urticarial dermatitis and prurigo 117
Urticaria and angioedema 117
Polymorphic eruption of pregnancy 120
Erythema annulare centrifugum 121
Wells syndrome 122
Eosinophilic annular erythema 124
Urticarial dermatitis 125
Shiitake dermatitis 126
Prurigo pigmentosa 127
Prurigo nodularis 129
Eruptive pseudoangiomatosis 131

Chapter 05
Erythema multiforme and graft-versus-host disease 133
Erythema multiforme 133
Graft-versus-host disease (GVHD) 136

Chapter 06
Drug-induced skin reaction 140
Drug eruption 140
Fixed drug eruption 144
Drug reaction with eosinophilia and systemic symptoms 145
Acute generalized exanthematous pustulosis 147
Stevens-Johnson syndrome and toxic epidermal necrolysis 149
Cutaneous adverse reactions associated with cancer targeted therapies 152
Hand-foot syndrome 154
Hand-foot skin reaction 155
Argyria 156

Chapter 07
Papulosquamous diseases 158
Psoriasis 158
Pustular psoriasis 162
Pityriasis rosea 164
Gianotti-Crosti syndrome 166
Lichen planus 167
Lichen planopilaris 172
Lichen planus pigmentosus 175
Lichen planus-like keratosis 176
Lichen nitidus 178
Lichen striatus 180
Inflammatory linear verrucous epidermal nevus 182
Pityriasis rubra pilaris 184
Small plaque parapsoriasis 187
Keratosis lichenoides chronica 188
Pityriasis lichenoides 189
Pityriasis rotunda 193
Circumscribed palmar or plantar hypokeratosis 194

Chapter 08
Bullous diseases 196
Pemphigus 196
IgA pemphigus 204
Paraneoplastic pemphigus 206
Bullous pemphigoid 209
Pemphigoid gestationis 212
Mucous membrane pemphigoid 213
Linear IgA dermatosis 214
Anti-p200 pemphigoid 216
Epidermolysis bullosa acquisita 217
Dermatitis herpetiformis 220
Transient acantholytic dermatosis 221
Subcorneal pustular dermatosis 222
Diabetic bulla 224
Erythema toxicum neonatorum 225

Chapter 09
Vasculitis and vascular diseases 227
Terminology of vascular disorders 227
Leukocytoclastic vasculitis 228
Urticarial vasculitis 231
Henoch-Schonlein purpura 232
Erythema elevatum diutinum 234
Cutaneous polyarteritis nodosa 236
ANCA-associated vasculitis 238
Purpura fulminans 242
Superficial thrombophlebitis 243
Thromboangiitis obliterans (Buerger’s disease) 245
Senile purpura 246
Pigmented purpuric dermatosis 247
Livedo reticularis 252
Livedoid vasculopathy 255
Nevus anemicus 257
Bier spots 259

Chapter 10
Neutrophilic dermatoses 260
Neutrophilic dermatoses 260
Behcet’s disease 260
Pyoderma gangrenosum 267
Sweet syndrome 269
Neutrophilic figurate erythema 271
Neutrophilic urticarial dermatosis 272

Chapter 11
Connective tissue diseases 274
Lupus erythematosus (LE) 274
Discoid lupus erythematosus (DLE) 275
Lupus erythematosus profundus/panniculitis 278
Lupus erythematosus tumidus 279
Chilblain lupus erythematosus 279
Subacute cutaneous lupus erythematosus (SCLE) 281
Neonatal lupus erythematosus 281
Cutaneous manifestations of systemic lupus erythematosus 282
Jessner’s lymphocytic infiltration of the skin 284
Sjogren’s syndrome 286
Dermatomyositis 287
Scleroderma 290
Systemic sclerosis 291
Morphea/Localized scleroderma 291
Lichen sclerosus 295
Atrophoderma of Pasini and Pierini 297

Chapter 12
Photosensitive diseases and
physical injury 298
Polymorphous light eruption 298
Hydroa vacciniforme 299
Chronic actinic dermatitis 300
Poikiloderma of Civatte 302
Burn 303
Erythema ab igne 305
Radiation dermatitis 306
Eosinophilic, polymorphic, and pruritic eruption associated with radiotherapy (EPPER) 307
Chilblain 308
Friction blister 310
Calcaneal petechiae 311
Bullae and sweat gland necrosis 312

Chapter 13
Noninfectious granuloma 313
Granuloma annulare 313
Interstitial granulomatous dermatitis 315
Rheumatoid nodules 317
Necrobiosis lipoidica 318
Sarcoidosis 319
Foreign body granuloma 323
Cheilitis granulomatosa 326

Chapter 14
Degenerative diseases 328
Solar elastosis 328
Reactive perforating collagenosis 330
Perforating folliculitis 331
Elastosis perforans serpinginosum 332
Kyrle disease 333
Striae distensa 334
Pseudoxanthoma elasticum like papillary dermal elastolysis 336
Anetoderma 337
Skin atrophy on the corticosteroid injection site 338
Diabetic dermopathy 339
Diabetic bulla 340
Diabetic gangrene 342

Chapter 15
Nutritional diseases 343
Pellagra 343
Necrolytic migratory erythema 344
Acrodermatitis enteropathica 345

Chapter 16
Metabolic diseases 347
Amyloidosis 347
AL amyloidosis (Primary systemic amyloidosis) 347
Lichen amyloidosis 349
Macular amyloidosis 351
Nodular localized cutaneous amyloidosis 352
Colloid milium 354
Lipoid proteinosis 355
Congenital erythropoietic porphyria (CEP) 356
Porphyria cutanea tarda (PCT) 358
Calcinosis cutis 359
Idiopathic calcinosis of the scrotum 360
Subepidermal calcified nodule 362
Gouty tophi 362
Pretibial myxedema 364
Cutaneous focal mucinosis 366
Acral persistent papular mucinosis 367
Scleredema 368
Acanthosis nigricans 369
Confluent and reticulated papillomatosis 371

Chapter 17
Inflammatory diseases of hair follicles 373
Acne 373
Acne keloidalis nuchae 375
Favre-Racouchot syndrome 377
Folliculitis 378
Bacterial folliculitis 378
Pityrosporum folliculitis 379
Eosinophilic pustular folliculitis (EPF) 381
Rosacea 383
Periorificial dermatitis 385
Lupus miliaris disseminatus faciei (LMDF) 387
Follicular occlusion tetrad 388
Hidradenitis suppurativa 388
Acne conglobata 391
Dissecting cellulitis 393
Pilonidal sinus 394
Folliculitis decalvans 395
Keratosis pilaris 397
Erythromelanosis follicularis faciei et colli 398
Keratosis follicularis squamosa (Dohi) 399

Chapter 18
Diseases of hair loss 400
Androgenetic alopecia 402
Alopecia areata 405
Telogen effluvium 408
Anagen effluvium 409
Trichotillomania 410
Alopecia mucinosa 414
Congenital triangular alopecia 415
Discoid lupus erythematosus 417
Lichen planopilaris 419
Frontal fibrosing alopecia 422
Pseudopelade of Brocq 424
Scleroderma 425
Folliculitis decalvans 427
Dissecting cellulitis 428

Chapter 19
Nail signs and disorders 430
Nail signs and nail specific conditions 431
Onycholysis 431
Nail pitting 432
Trachyonychia 432
Brittle nails 433
Beau lines 434
Onychomadesis 434
Retronychia 435
Ingrowing nails 437
Pincer nail 437
Onychogryposis 438
Pterygium 439
Median canaliform nail dystrophy 440
Chromonychia (Nail discolarations) 441
Chromonychia (Nail discolarations) 441
Leukonychia 441
Erythronychia 443
Melanonychia 444
Subungual hematoma 445
Splinter hemorrhages 446
Inflammatory nail diseases 447
Psoriatic nails 447
Alopecia areata 447
Lichen planus 447
Pemphigus 448
Connective tissue diseases 448
Chronic paronychia 448
Infectious nail diseases 450
Onychomycosis 450
Green nail syndrome 451
Traumatic nail disorders 452
Onychotillomania (nail-picking disorder) 452
Onychophagia (fingernail biting) 452
Habit-tic nail deformity 452
Habit tic deformity 452
Nail tumors 454
Onychomatricoma 454
Onychopapilloma 455
Acquired ungual fibrokeratoma 456
Glomus tumor 457
Digital mucous cyst 458
Subungual exostosis 459

Chapter 20
Inflammatory diseases of the subcutaneous fat 461
Erythema nodosum 461
Erythema Induratum 464
Localized idiopathic lipodystrophy 466
Centrifugal abdominal lipodystrophy 466
Semicircular lipoatrophy 467
Lipodermatosclerosis 468
Traumatic panniculitis 470
Nodular cystic fat necrosis 472
Subcutaneous fat necrosis of the newborn 474
Sclerema neonatorum 475

Chapter 21
Bacterial infections 476
Impetigo contagiosa 476
Bullous impetigo 477
Staphylococcal scalded skin syndrome 479
Ecthyma 480
Multiple sweat gland abscesses 481
Cellulitis/Erysipelas 482
Superficial folliculitis 484
Hot-tub folliculitis 485
Furuncles, carbuncles, and abscesses 487
Acute paronychia 488
Fournier’s gangrene 489
Pitted keratolysis 490
Trichomycosis axillaris 491
Tsutsugamushi disease 492
Lyme disease 494
Tuberculoid leprosy 495
Lepromatous leprosy 497
Lupus vulgaris 499
Scrofuloderma 500
Tuberculosis verrucosa cutis 501
Papulonecrotic tuberculid 502
Mycobacterium marinum infection 504
Mycobacterium fortuitum infection 506
Secondary syphilis 509
Congenital syphilis 512
Black hairy tongue 513

Chapter 22
Viral Infections 515
Herpes simplex 516
Eczema herpeticum 518
Varicella 519
Herpes zoster 520
Molluscum contagiosum 522
Verruca vulgaris 523
Verruca plantaris 525
Verruca plana 526
Epidermodyslpasia verruciformis 527
Condyloma acuminata 529
Bowenoid papulosis 530
Hand-foot-mouth disease 531
Erythema infectiosum 532

Chapter 23
Fungal infections 534
Dermatophytoses 535
Kerion celsi 539
Majocchi’s granuloma 540
Malassezia infection 542
Pityriasis versicolor 542
Malassezia folliculitis 544
Candidiasis 546
Sporotrichosis 547
Chromoblastomycosis 548
Protothecosis 550

Chapter 24
Parasites and protozoa infestations 552
Insect bite 552
Bee-sting granuloma 555
Scabies 556
Tsutsugamushi disease 559
Pediculosis 560
Tick bite 561
Cutaneous larva migrans 563
Sparganosis 565
Cutaneous leishmaniasis 566

Chapter 25
Histiocytosis 568
Langerhans cell histiocytosis (LCH) 569
Juvenile xanthogranuloma (JXG) 572
Xanthoma disseminatum 575
Rosai-Dorfman disease 576
Multicentric reticulohistiocytosis 578
Xanthomas 580
Xanthelasma 582
Verruciform xanthoma 583
Mastocytoma 584
Primary cutaneous plasmacytoma 586

Chapter 26
Pigmentary disorders 587
Postinflammatory hyperpigmentation (PIH) 588
Melasma 589
Pigmented contact dermatitis 591
Lichen planus pigmentosus (LPP) 593
Idiopathic eruptive macular pigmentation 594
Erythema dyschromicum perstans 596
Confluent and reticulated papillomatosis 597
Linear and whorled nevoid hypermelanosis 599
Progressive cribriform and zosteriform hyperpigmentation 600
Addison’s disease 601
Piebaldism 602
Vitiligo 603
Halo nevus 606
Nevus depigmentosus 607
Progressive macular hypomelanosis 608
Idiopathic guttate hypomelanosis 610
Hypomelanosis of Ito 611
Pityriasis alba 612
Reticulate acropigmentation of Kitamura 614
Dyschromatosis symmetrica hereditaria 615
Dyschromatosis universalis hereditaria 616

Chapter 27
Benign melanocytic tumors and proliferations 618
Freckles 618
Mucosal melanotic macule 619
Solar lentigo 621
Lentigo simplex 622
Familial lentiginosis syndromes 624
Partial unilateral lentiginosis 624
Nevus spilus 625
Becker’s nevus 626
Pigmented fungiform papillae of tongue 628
Cafe-au-lait macules 629
Mongolian spots 630
Nevus of Ota 631
Nevus of Ito 632
Acquired bilateral nevus of Ota-like macules (ABNOM) 633
Dermal melanocytic hamartoma 634
Phakomatosis pigmentovascularis 635
Blue nevus 636
Melanocytic nevus 638
Junctional nevus 640
Compound nevus 642
Intradermal nevus 644
Halo nevus 648
Congenital melanocytic nevus 649
Spitz nevus 651
Pigmented spindle cell nevus 653
Dysplastic nevus 654
Melanonychia 656

Chapter 28
Malignant melanoma 658
Malignant melanoma 658
Melanoma of the nail unit 666
Nevoid melanoma 668
Mucosal and uveal melanoma 669

Chapter 29
Benign tumors and cysts of the epidermis 671
Linear epidermal nevus 671
Epidermal nevus with epidermolytic hyperkeratosis 673
Acrokeratosis verruciformis of Hopf 674
Nevoid hyperkeratosis of nipple and areola 675
Nevus comedonicus 675
Seborrheic keratosis 676
Inverted follicular keratosis 682
Stucco keratosis 684
Clear cell acanthoma 684
Warty dyskeratoma 685
Oral leukoplakia 686
Epidermal cyst 687
Pilar cyst 689
Proliferating trichilemmal cyst 690
Steatocystoma multiplex 691
Eruptive vellus hair cyst 693
Median raphe cyst 694
Bronchogenic cyst 694
Branchial cleft cyst 696
Pseudocyst of the auricle 697

Chapter 30
Malignant tumors of the epidermis 698
Actinic keratosis (AK) 698
Actinic cheilitis 701
Bowen’s disease 702
Erythrodysplasia of Queyrat 705
Arsenical keratosis 706
Squamous cell carcinoma (SCC) 707
Verrucous carcinoma 714
Keratoacanthoma (KA) 715
Basal cell carcinoma (BCC) 717
Mammary Paget’s disease (MPD) 722
Extramammary Paget’s disease (EMPD) 724

Chapter 31
Tumors of the epidermal appendages 726
Tumors of hair differentiation 726
Hair follicle nevus 726
Dilated pore of Winer 727
Basaloid follicular hamartoma 729
Trichofolliculoma 730
Trichoadenoma 732
Trichilemmoma 733
Trichilemmal carcinoma 735
Pilar sheath acanthoma 737
Proliferating trichilemmal tumor 738
Pilomatricoma 740
Fibrofolliculoma 742
Birt-Hogg-Dube syndrome 743
Trichoepithelioma 744
Desmoplastic trichoepithelioma 746
Trichoblastoma 748
Tumors of sebaceous gland differentiation 750
Ectopic sebaceous glands 750
Juxta-clavicular beaded lines 751
Sebaceous hyperplasia 752
Nevus sebaceous 754
Superficial epithelioma with sebaceous differentiation 756
Sebaceous adenoma 757
Sebaceous epithelioma 759
Sebaceous carcinoma 760
Muir-Torre syndrome 762
Tumors of eccrine and apocrine gland differentiation 764
Eccrine nevus 764
Eccrine angiomatous hamartoma 765
Eccrine hydrocystoma 766
Syringoma 768
Eccrine poroma 770
Hidroacanthoma simplex 772
Eccrine porocarcinoma 774
Eccrine spiradenoma 775
Cylindroma 777
Papillary eccrine adenoma 779
Nodular hidradenoma 780
Chondroid syringoma 783
Mucinous eccrine carcinoma 786
Apocrine hidrocystoma 787
Syringocystadenoma papilliferum 788
Microcystic adnexal carcinoma (MAC) 791
Primary cutaneous apocrine carcinoma 793

Chapter 32
Cutaneous lymphoma, leukemia and other myeloproliferative disorders 796
Mycosis fungoides (MF) 796
Folliculotrophic mycosis fungoides 808
Sezary syndrome 810
Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) 813
Lymphomatoid papulosis (LyP) 816
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) 819
Extranodal NK/T-cell lymphoma, nasal type (ENKL) 822
Primary cutaneous γ/δ T-cell lymphoma 824
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder 826
Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PCPTCL-NOS) 828
Marginal zone B cell lymphoma (PCMZL) 830
Primary cutaneous follicle center lymphoma (PCFCL) 833
Primary cutneous diffuse large B cell lymphoma, leg-type (PCDLBL-LT) 835
Leukemia cutis 837
Burkitt lymphoma 840
Cutaneous pseudolymphoma 842
Kimura’s disease 845

Chapter 33
Tumors of fibrous tissue 847
Fibrohistiocytic tumors 847
Definition of fibrohistiocytic tumors 847
Dermatofibroma 847
Cellular dermatofibroma 850
Aneurysmal dermatofibroma 851
Epithelioid dermatofibroma 852
Atypical dermatofibroma 854
Giant cell tumor of tendon sheath 855
Dermatofibrosarcoma protuberans (DFSP) 857
Bednar tumor 859
Giant cell fibroblastoma 860
Atypical fibroxanthoma 861
Myxofibrosarcoma 863
Low grade fibromyxoid sarcoma (LGFMS) 864
Fibroblastic/myofibroblastic tumors 866
Definition of fibroblastic and myofibroblastic tumors 866
Hypertrophic scar 866
Keloid 868
Soft fibroma 869
Fibroepithelial polyp of the anus 871
Sclerotic fibroma 872
Fibroma of tendon sheath 873
Nodular fasciitis 875
Myofibroma 876
Angiofibroma 878
Cemento-ossifying fibroma 882
Nuchal fibroma 883
Superficial acral fibromyxoma 884
Palmar and plantar fibromatosis 886
Knuckle pad 887
Solitary fibrous tumor 888
Miscellaneous lesions 889
Cutaneous myxoma 890
Digital mucous cyst 891
Ganglion cyst 892
Oral mucous cyst 893
White fibrous papulosis of the neck 895
Corn 896

Chapter 34
Vascular tumors and malformations 898
ISSVA classification of vascular anomalies 898
Infantile hemangioma 899
Congenital hemangioma 901
Tufted angioma 902
Epithelioid hemangioma 905
Pyogenic granuloma 906
Hobnail hemangioma 908
Microvenular hemangioma 910
Glomeruloid hemangioma 911
Intravascular papillary endothelial hyperplasia 912
Kaposi sarcoma 914
Angiosarcoma 917
Nevus flammeus 920
Generalized essential telangiectasia 923
Unilateral nevoid telangiectasia 924
Acquired bilateral telangiectatic macules 925
Angiokeratoma 927
Cherry hemangioma 931
Lymphatic malformation 933
Venous malformations 937
Blue rubber bleb nevus syndrome 940
Verrucous venous malformation 941
Venous lake 943
Capillary aneurysm 944
Arteriovenous hemangioma 946
Sinusoidal hemangioma 947
Arteriovenous malformation 948
Acquired arteriovenous malformation 950
Glomus tumor 951
Myopericytoma 955

Chapter 35
Tumors of adipose tissue, muscle, cartilage and bone 958
Nevus lipomatosus superficialis 958
Lipoma 959
Angiolipoma 961
Spindle cell lipoma 962
Sclerotic lipoma 964
Well-differentiated liposarcoma 965
Smooth muscle hamartoma 966
Rhabdomyomatous mesenchymal hamartoma 967
Leiomyoma 968
Cutaneous leiomyosarcoma 973
True cutaneous chondroma 974
Osteoma cutis 974
Subungual exostosis 975

Chapter 36
Tumors of neural tissue 977
Neurofibromas 977
Neurofibromatosis type 1 979
Segmental neurofibromatosis 980
Cutaneous lipomatous neurofibroma 981
Intraneural neurofibroma 982
Plexiform neurofibroma 983
Palisaded encapsulated neuroma 985
Sclerosing perineurioma 986
Schwannoma 987
Plexiform schwannoma 990
Granular cell tumor 991
Merkel cell carcinoma 992

Chapter 37
Metastatic carcinomas of the skin 995
Cutaneous metastatic breast carcinoma 995
Cutaneous metastatic lung carcinoma 998
Cutaneous metastatic stomach cancer 1002
Cutaneous metastatic colorectal carcinoma 1003
Cutaneous metastasis from pancreatic cancer 1004
Cutaneous metastasis from oral squamous cell
carcinoma 1005
Cutaneous metastasis from thyroid cancer 1006
Cutaneous metastasis from renal cell carcinoma 1006
Cutaneous metastasis from ovarian cancer 1007

Chapter 38
Ectopic tissues and miscellaneous tumors 1008
Accessory tragus 1008
Supernumerary nipple 1009
Accessory breast 1011
Cutaneous endometriosis 1012
Canalicular adenoma 1014
Pleomorphic adenoma 1015
Nipple adenoma 1017
Intraductal papilloma 1018
Oral cutaneous fistula 1019