Preface xii
1 Introduction 1
Classification of cutaneous lymphomas 1
Examination of patients 2
Staging investigations 2
Surgical techniques 2
Histopathology immunophenotype and molecular genetics 4
Other methods used in the study of cutaneous lymphoid infiltrates 11
Lymphoma microenvironment and lymphoma- associated microorganisms 11
Pseudomalignancy premalignancy and early malignancy 12
Section 1: Cutaneous NK/T-cell lymphomas and lymphoproliferative disorders
2 The “parapsoriases” 17
3 Mycosis fungoides 26
Onset of mycosis fungoides or exacerbation of undiagnosed disease under treatment with immunomodulatory agents 28
Clinical features 29
Extracutaneous involvement 34
Association with other diseases 37
Histopathology immunophenotype and molecular genetics 39
Histopathologic differential diagnosis from inflammatory conditions 61
Clinical and histopathologic variants 62
Mycosis fungoides in children and adolescents 99
Treatment 100
Prognosis 111
4 Sézary syndrome 135
Onset/progression under systemic therapies for benign inflammatory dermatoses 137
Clinical features 137
Histopathology immunophenotype and molecular genetics 145
Treatment 149
Prognosis 151
5 Primary cutaneous CD30 + lymphoproliferative disorders 158
Lymphomatoid Papulosis 159
Cutaneous Anaplastic Large Cell Lymphoma 180
“Borderline” Cases 198
Intralymphatic CD30 + Large T- Cell Lymphoma 199
Implant- associated anaplastic large cell lymphoma 202
6 Subcutaneous panniculitis- like T- cell lymphoma 211
Clinical features 212
Histopathology immunophenotype and molecular genetics 214
Differential diagnosis with other cutaneous NK/T- cell lymphomas with prominent involvement of the subcutaneous tissue 218
Differential diagnosis with lupus panniculitis 218
Treatment 219
Prognosis 223
7 Primary cutaneous aggressive epidermotropic CD8 + cytotoxic T- Cell lymphoma 227
Clinical features 227
Histopathology immunophenotype and molecular genetics 227
Treatment 233
Prognosis 236
8 Primary cutaneous γ/δ T- cell lymphoma 240
Clinical features 242
Histopathology immunophenotype and molecular genetics 243
Prognosis 248
9 Cutaneous extranodal NK/T- cell lymphoma nasal type 254
Clinical features 254
Histopathology immunophenotype and molecular genetics 256
Treatment 262
Prognosis 263
10 Cutaneous peripheral T- cell lymphoma not otherwise specified (NOS) 266
Clinical features 267
Histopathology immunophenotype and molecular genetics 268
Treatment 272
Prognosis 272
11 Cutaneous CD4 + small– medium T- cell lymphoproliferative disorder 278
Clinical features 280
Histopathology immunophenotype and molecular genetics 281
Treatment 284
Prognosis 284
Final considerations 284
12 Primary cutaneous acral CD8 + T- cell lymphoproliferative disorder 287
Clinical features 287
Histopathology immunophenotype and molecular genetics 287
Treatment 288
Prognosis 288
13 Cutaneous adult T- cell leukemia/lymphoma 291
Clinical features 291
Histopathology 291
Immunophenotype 291
Molecular genetics 293
Treatment 293
Prognosis 293
14 Specific cutaneous manifestations of nodal T- follicular helper cell lymphoma angioimmunoblastic type and of other nodal T- follicular helper cell lymphomas 296
Clinical features 296
Histopathology 296
Immunophenotype 299
Molecular genetics 299
Treatment 299
Prognosis 299
15 Cutaneous manifestations in Epstein– Barr virus- positive T- cell and NK- cell lymphoid proliferations and lymphomas of childhood 304
Severe mosquito bite allergy 304
Hydroa- vacciniforme lymphoproliferative disorders 305
Classic hydroa vacciniforme 306
Systemic hydroa vacciniforme lymphoproliferative disorder 309
Section 2: Cutaneous B-cell lymphomas and lymphoproliferative disorders
16 Cutaneous follicle center lymphoma 317
Clinical features 317
Histopathology immunophenotype and molecular genetics 321
Differential diagnosis of cutaneous follicle center lymphoma diffuse type from cutaneous diffuse large B- cell lymphoma leg type 336
Treatment 336
Prognosis 337
17 Primary cutaneous marginal zone lymphoproliferative disorder (primary cutaneous marginal zone lymphoma) and variants 341
Cutaneous marginal zone lymphoproliferative disorder conventional variant 343
Clinical features 344
Histopathology immunophenotype and molecular genetics 345
Treatment 354
Prognosis 355
Cutaneous marginal zone lymphoproliferative disorder lymphoplasmacytic variant 356
Clinical features 356
Histopathology immunophenotype and molecular genetics 356
Treatment 358
Prognosis 358
Cutaneous marginal zone lymphoproliferative disorder plasmacytic variant 359
Clinical features 359
Histopathology immunophenotype and molecular genetics 359
Treatment and prognosis 359
Cutaneous marginal zone lymphoproliferative disorder blastoid variant 361
Clinical features 361
Histopathology immunophenotype and molecular genetics 361
Treatment and prognosis 362
Cutaneous amyloidoma 362
Clinical features 364
Histopathology immunophenotype and molecular genetics 364
Treatment and prognosis 364
18 Cutaneous diffuse large B- cell lymphoma leg type 370
Clinical features 371
Histopathology immunophenotype and molecular genetics 372
Treatment 380
Prognosis 380
Primary cutaneous diffuse large B- cell lymphoma Nos 381
19 Intravascular large cell lymphomas 387
Intravascular large B- cell lymphoma 388
Intravascular large NK/T- cell lymphoma 394
20 Other cutaneous B- cell lymphomas 400
Cutaneous manifestations of Epstein– Barr virus (EBV)- positive diffuse large B- cell lymphoma 400
Cutaneous manifestations of mantle cell lymphoma 403
Cutaneous manifestations of extracavitary primary effusion lymphoma 408
Cutaneous manifestations of multiple myeloma 411
Cutaneous manifestations of Burkitt lymphoma 414
Cutaneous lymphomatoid granulomatosis 416
Cutaneous plasmablastic lymphoma 419
Cutaneous T- cell/histiocyte- rich large B- cell lymphoma 420
Cutaneous manifestations of Waldenström macroglobulinemia 424
Section 3: Cutaneous lymphomas and lymphoproliferative disorders in the setting of immune deficiency / dysregulation
21 Cutaneous lymphoproliferative disorders and lymphomas in the setting of immune deficiency/ dysregulation 433
Posttransplant immunodeficiency/dysregulation 439
Human immunodeficiency virus (HIV)- related immune deficiency/dysregulation 439
Cutaneous plasmacytic hyperplasia associated with immune deficiency/dysregulation 441
Cutaneous polymorphic B- cell lymphoproliferative disorder associated with immune deficiency/ dysregulation 443
Epstein– Barr virus (EBV)- associated mucocutaneous ulcers 446
Cutaneous manifestations of multicentric Castleman disease 448
Section 4: Specific cutaneous manifestations of leukemias and of precursor hematologic neoplasms
22 Cutaneous manifestations of B- cell chronic lymphocytic leukemia 455
Clinical features 455
Histopathology immunophenotype and molecular genetics 456
Treatment and prognosis 461
Progression to diffuse large B- cell lymphoma (Richter syndrome) 464
23 Cutaneous manifestations of myelogenous leukemia 471
Acute myeloid leukemia 472
Chronic myelogenous leukemia 484
Myelodysplastic syndromes (myelodysplastic neoplasms) 489
Mature plasmacytoid dendritic cell proliferation associated with myeloid neoplasms 492
24 Blastic plasmacytoid dendritic cell neoplasm 495
Clinical features 495
Histopathology immunophenotype and molecular genetics 497
Treatment 502
Prognosis 503
25 Cutaneous manifestations of other leukemias 507
T- cell prolymphocytic leukemia 507
Aggressive natural killer cell leukemia 509
Specific skin manifestations of other types of leukemia 510
26 Cutaneous lymphoblastic lymphomas 513
Cutaneous B- Lymphoblastic Lymphoma 513
Clinical features 514
Histopathology immunophenotype and molecular genetics 514
Differential diagnosis 516
Treatment and prognosis 517
Cutaneous T- Lymphoblastic Lymphoma 518
Clinical features 518
Histopathology immunophenotype and molecular genetics 519
Treatment and prognosis 519
Section 5: Cutaneous manifestations of other lymphomas and non-neoplastic cutaneous signs of systemic lymphomas
27 Cutaneous manifestations of Hodgkin lymphoma 527
Clinical features 528
Histopathology immunophenotype and molecular genetics 528
Treatment and prognosis 530
Primary cutaneous Hodgkin lymphoma 530
28 Cutaneous composite lymphomas 533
Clinical features 534
Histopathology immunophenotype and molecular genetics 534
Treatment and prognosis 538
29 Non- neoplastic cutaneous signs of systemic lymphomas 541
Section 6: Cutaneous lymphomas and lymphoproliferative disorders in children and adolescents
30 Cutaneous lymphomas and lymphoproliferative disorders in children and adolescents 599
Mycosis fungoides 599
Section 7: Pseudolymphomas of the skin
31 Pseudolymphomas of the skin 577
Chronic actinic dermatitis (actinic reticuloid) 578
Lymphomatoid contact dermatitis 580
Pseudolymphomatous atopic dermatitis and other eczematous dermatoses 582
Non- neoplastic erythroderma in adult patients 592
Solitary idiopathic T/B- cell pseudolymphoma 598
Lichenoid/lymphomatoid keratosis 600
Pityriasis lichenoides 601
Lichen aureus/lichenoid pigmented purpuric dermatitis 605
Lichen sclerosus 606
Vitiligo (inflammatory stage) 609
Annular lichenoid dermatitis of youth 609
Pseudolymphomatous cutaneous infiltrates in human immunodeficiency virus (HIV)- infected patients and in other immune deficiencies 609
CD30 + T- cell pseudolymphomas 614
Persistent nodular arthropod bite reactions and nodular scabies 616
Pseudolymphomas in herpes simplex and herpes zoster infections 620
Pseudolymphomas associated with lupus erythematosus 621
Lymphomatoid drug reactions 630
Lymphocytoma cutis 633
Pseudolymphomas associated with infections by Borrelia species 635
Pseudolymphomas at sites of vaccination 642
Localized scleroderma/morphea 647
Cutaneous IgG4- related disease 649
Pseudolymphomas in syphilis 649
Cutaneous plasma cell granuloma 654
Cutaneous and systemic plasmacytosis 655
Angiomatoid pseudolymphoma (T- cell- rich angiomatoid polypoid pseudolymphoma / Acral pseudolymphomatous angiokeratoma in children / Pre- tibial lymphoplasmacytic plaque in children) 655
Cutaneous extramedullary hematopoiesis 658
Histiocytoid cells in Sweet syndrome and other conditions 659
Intralymphatic histiocytosis 661
Benign intralymphatic proliferation of T- cell lymphoid blasts 662
Other “accidental” pseudolymphomas 667
32 Nonlymphoid malignant neoplasms of the skin mimicking histopathologically cutaneous lymphomas (“malignant pseudolymphomas”) 677
Section 8: The cutaneous “atypical lymphoid proliferation”
33 The cutaneous “atypical lymphoid proliferation” 687
Index 690
