Skin Lymphoma

Lorenzo Cerroni is the Director of the Dermatopathology Unit at the Medical University of Graz, Austria. He is the past President of the International Society of Dermatopathology and a recipient of numerous prestigious dermatology and dermatopathology awards.

• Preface xii1 Introduction 1Classification of cutaneous lymphomas 1Examination of patients 2Staging investigations 2Surgical techniques 2Histopathology immunophenotype and molecular genetics 4Other methods used in the study of cutaneous lymphoid infiltrates 11Lymphoma microenvironment and lymphoma- associated microorganisms 11Pseudomalignancy premalignancy and early malignancy 12Section 1: Cutaneous NK/T-cell lymphomas and lymphoproliferative disorders2 The “parapsoriases” 173 Mycosis fungoides 26Onset of mycosis fungoides or exacerbation of undiagnosed disease under treatment with immunomodulatory agents 28Clinical features 29Extracutaneous involvement 34Association with other diseases 37Histopathology immunophenotype and molecular genetics 39Histopathologic differential diagnosis from inflammatory conditions 61Clinical and histopathologic variants 62Mycosis fungoides in children and adolescents 99Treatment 100Prognosis 1114 Sézary syndrome 135Onset/progression under systemic therapies for benign inflammatory dermatoses 137Clinical features 137Histopathology immunophenotype and molecular genetics 145Treatment 149Prognosis 1515 Primary cutaneous CD30 + lymphoproliferative disorders 158Lymphomatoid Papulosis 159Cutaneous Anaplastic Large Cell Lymphoma 180“Borderline” Cases 198Intralymphatic CD30 + Large T- Cell Lymphoma 199Implant- associated anaplastic large cell lymphoma 2026 Subcutaneous panniculitis- like T- cell lymphoma 211Clinical features 212Histopathology immunophenotype and molecular genetics 214Differential diagnosis with other cutaneous NK/T- cell lymphomas with prominent involvement of the subcutaneous tissue 218Differential diagnosis with lupus panniculitis 218Treatment 219Prognosis 2237 Primary cutaneous aggressive epidermotropic CD8 + cytotoxic T- Cell lymphoma 227Clinical features 227Histopathology immunophenotype and molecular genetics 227Treatment 233Prognosis 2368 Primary cutaneous γ/δ T- cell lymphoma 240Clinical features 242Histopathology immunophenotype and molecular genetics 243Prognosis 2489 Cutaneous extranodal NK/T- cell lymphoma nasal type 254Clinical features 254Histopathology immunophenotype and molecular genetics 256Treatment 262Prognosis 26310 Cutaneous peripheral T- cell lymphoma not otherwise specified (NOS) 266Clinical features 267Histopathology immunophenotype and molecular genetics 268Treatment 272Prognosis 27211 Cutaneous CD4 + small– medium T- cell lymphoproliferative disorder 278Clinical features 280Histopathology immunophenotype and molecular genetics 281Treatment 284Prognosis 284Final considerations 28412 Primary cutaneous acral CD8 + T- cell lymphoproliferative disorder 287Clinical features 287Histopathology immunophenotype and molecular genetics 287Treatment 288Prognosis 28813 Cutaneous adult T- cell leukemia/lymphoma 291Clinical features 291Histopathology 291Immunophenotype 291Molecular genetics 293Treatment 293Prognosis 29314 Specific cutaneous manifestations of nodal T- follicular helper cell lymphoma angioimmunoblastic type and of other nodal T- follicular helper cell lymphomas 296Clinical features 296Histopathology 296Immunophenotype 299Molecular genetics 299Treatment 299Prognosis 29915 Cutaneous manifestations in Epstein– Barr virus- positive T- cell and NK- cell lymphoid proliferations and lymphomas of childhood 304Severe mosquito bite allergy 304Hydroa- vacciniforme lymphoproliferative disorders 305Classic hydroa vacciniforme 306Systemic hydroa vacciniforme lymphoproliferative disorder 309Section 2: Cutaneous B-cell lymphomas and lymphoproliferative disorders16 Cutaneous follicle center lymphoma 317Clinical features 317Histopathology immunophenotype and molecular genetics 321Differential diagnosis of cutaneous follicle center lymphoma diffuse type from cutaneous diffuse large B- cell lymphoma leg type 336Treatment 336Prognosis 33717 Primary cutaneous marginal zone lymphoproliferative disorder (primary cutaneous marginal zone lymphoma) and variants 341Cutaneous marginal zone lymphoproliferative disorder conventional variant 343Clinical features 344Histopathology immunophenotype and molecular genetics 345Treatment 354Prognosis 355Cutaneous marginal zone lymphoproliferative disorder lymphoplasmacytic variant 356Clinical features 356Histopathology immunophenotype and molecular genetics 356Treatment 358Prognosis 358Cutaneous marginal zone lymphoproliferative disorder plasmacytic variant 359Clinical features 359Histopathology immunophenotype and molecular genetics 359Treatment and prognosis 359Cutaneous marginal zone lymphoproliferative disorder blastoid variant 361Clinical features 361Histopathology immunophenotype and molecular genetics 361Treatment and prognosis 362Cutaneous amyloidoma 362Clinical features 364Histopathology immunophenotype and molecular genetics 364Treatment and prognosis 36418 Cutaneous diffuse large B- cell lymphoma leg type 370Clinical features 371Histopathology immunophenotype and molecular genetics 372Treatment 380Prognosis 380Primary cutaneous diffuse large B- cell lymphoma Nos 38119 Intravascular large cell lymphomas 387Intravascular large B- cell lymphoma 388Intravascular large NK/T- cell lymphoma 39420 Other cutaneous B- cell lymphomas 400Cutaneous manifestations of Epstein– Barr virus (EBV)- positive diffuse large B- cell lymphoma 400Cutaneous manifestations of mantle cell lymphoma 403Cutaneous manifestations of extracavitary primary effusion lymphoma 408Cutaneous manifestations of multiple myeloma 411Cutaneous manifestations of Burkitt lymphoma 414Cutaneous lymphomatoid granulomatosis 416Cutaneous plasmablastic lymphoma 419Cutaneous T- cell/histiocyte- rich large B- cell lymphoma 420Cutaneous manifestations of Waldenström macroglobulinemia 424Section 3: Cutaneous lymphomas and lymphoproliferative disorders in the setting of immune deficiency / dysregulation21 Cutaneous lymphoproliferative disorders and lymphomas in the setting of immune deficiency/ dysregulation 433Posttransplant immunodeficiency/dysregulation 439Human immunodeficiency virus (HIV)- related immune deficiency/dysregulation 439Cutaneous plasmacytic hyperplasia associated with immune deficiency/dysregulation 441Cutaneous polymorphic B- cell lymphoproliferative disorder associated with immune deficiency/ dysregulation 443Epstein– Barr virus (EBV)- associated mucocutaneous ulcers 446Cutaneous manifestations of multicentric Castleman disease 448Section 4: Specific cutaneous manifestations of leukemias and of precursor hematologic neoplasms22 Cutaneous manifestations of B- cell chronic lymphocytic leukemia 455Clinical features 455Histopathology immunophenotype and molecular genetics 456Treatment and prognosis 461Progression to diffuse large B- cell lymphoma (Richter syndrome) 46423 Cutaneous manifestations of myelogenous leukemia 471Acute myeloid leukemia 472Chronic myelogenous leukemia 484Myelodysplastic syndromes (myelodysplastic neoplasms) 489Mature plasmacytoid dendritic cell proliferation associated with myeloid neoplasms 49224 Blastic plasmacytoid dendritic cell neoplasm 495Clinical features 495Histopathology immunophenotype and molecular genetics 497Treatment 502Prognosis 50325 Cutaneous manifestations of other leukemias 507T- cell prolymphocytic leukemia 507Aggressive natural killer cell leukemia 509Specific skin manifestations of other types of leukemia 51026 Cutaneous lymphoblastic lymphomas 513Cutaneous B- Lymphoblastic Lymphoma 513Clinical features 514Histopathology immunophenotype and molecular genetics 514Differential diagnosis 516Treatment and prognosis 517Cutaneous T- Lymphoblastic Lymphoma 518Clinical features 518Histopathology immunophenotype and molecular genetics 519Treatment and prognosis 519Section 5: Cutaneous manifestations of other lymphomas and non-neoplastic cutaneous signs of systemic lymphomas27 Cutaneous manifestations of Hodgkin lymphoma 527Clinical features 528Histopathology immunophenotype and molecular genetics 528Treatment and prognosis 530Primary cutaneous Hodgkin lymphoma 53028 Cutaneous composite lymphomas 533Clinical features 534Histopathology immunophenotype and molecular genetics 534Treatment and prognosis 53829 Non- neoplastic cutaneous signs of systemic lymphomas 541Section 6: Cutaneous lymphomas and lymphoproliferative disorders in children and adolescents30 Cutaneous lymphomas and lymphoproliferative disorders in children and adolescents 599Mycosis fungoides 599Section 7: Pseudolymphomas of the skin31 Pseudolymphomas of the skin 577Chronic actinic dermatitis (actinic reticuloid) 578Lymphomatoid contact dermatitis 580Pseudolymphomatous atopic dermatitis and other eczematous dermatoses 582Non- neoplastic erythroderma in adult patients 592Solitary idiopathic T/B- cell pseudolymphoma 598Lichenoid/lymphomatoid keratosis 600Pityriasis lichenoides 601Lichen aureus/lichenoid pigmented purpuric dermatitis 605Lichen sclerosus 606Vitiligo (inflammatory stage) 609Annular lichenoid dermatitis of youth 609Pseudolymphomatous cutaneous infiltrates in human immunodeficiency virus (HIV)- infected patients and in other immune deficiencies 609CD30 + T- cell pseudolymphomas 614Persistent nodular arthropod bite reactions and nodular scabies 616Pseudolymphomas in herpes simplex and herpes zoster infections 620Pseudolymphomas associated with lupus erythematosus 621Lymphomatoid drug reactions 630Lymphocytoma cutis 633Pseudolymphomas associated with infections by Borrelia species 635Pseudolymphomas at sites of vaccination 642Localized scleroderma/morphea 647Cutaneous IgG4- related disease 649Pseudolymphomas in syphilis 649Cutaneous plasma cell granuloma 654Cutaneous and systemic plasmacytosis 655Angiomatoid pseudolymphoma (T- cell- rich angiomatoid polypoid pseudolymphoma / Acral pseudolymphomatous angiokeratoma in children / Pre- tibial lymphoplasmacytic plaque in children) 655Cutaneous extramedullary hematopoiesis 658Histiocytoid cells in Sweet syndrome and other conditions 659Intralymphatic histiocytosis 661Benign intralymphatic proliferation of T- cell lymphoid blasts 662Other “accidental” pseudolymphomas 66732 Nonlymphoid malignant neoplasms of the skin mimicking histopathologically cutaneous lymphomas (“malignant pseudolymphomas”) 677Section 8: The cutaneous “atypical lymphoid proliferation”33 The cutaneous “atypical lymphoid proliferation” 687Index 690