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.