586 Annular lichenoid dermatitis clinical features, 151 diagnosis, 152 epidermotropic cells, 152, 153 genetics and molecular findings, 151 histopathol

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1 Index A Acquired immune deficiency syndrome (AIDS) bacillary angiomatosis, 423, 424 CD4+ cells, 423 differential diagnosis, 423 EBV infection, 423 etiology and epidemiology, herpesviruses and human papillomavirus, 423 histopathology, 423 HIV-infected individuals, 423 Kaposi s sarcoma, 423 North America and Europe, 403 oral hairy leukoplakia, 423, 424 pandemic of, 405 Actinic reticuloid (AR) chronic actinic dermatitis, 236 clinical course, 239 cytomorphology, 237 diagnosis, 161, differential diagnosis, 137 epidemiology, 237 features, 160 genetics and molecular findings, 161, 239 histopathology, 160 Hodgkin s lymphoma, 237 immunophenotype, , 238, 239 infiltration pattern, 237, 238 lesions, 237 and MF, 161 prognosis and clinical course, 161 reactive erythroderma, Addressins, 26, 32 ALCL. See Anaplastic large cell lymphoma (ALCL) ALK. See Anaplastic lymphoma kinase gene (ALK) ALLP. See Atypical lymphocytic lobular panniculitis (ALLP) Alopecia chronic hepatitis C infection, 480 rubbing, scalp, 160 cutaneous manifestations, 415 interferon and ribavirin, 478 syringolymphoid hyperplasia, 114 Amelanotic melanoma, Anaplastic large cell lymphoma (ALCL) calcl, 194, 351, 352, 370, 545, 556 CD30+ tumor cells, 326, 328 and LyP, myxoid, 351 NK-/T-cell lymphoma, 530, 531 primary (see Primary cutaneous ALCL) Sézary syndrome (SS), 194 systemic, 109, 254, 356 Anaplastic lymphoma kinase gene (ALK) CD246, 35 CD30 expression, 69, 182 cytoplasmic activity, 336, , 356 and EMA, 69 gene rearrangement, 224 lack of, 356 nonlymphoid tumors, 551, 568, 576 nucleophosmin, oncoprotein, 202 single cutaneous, 69 Angioendotheliomatosis, 521, 530, 533 Angioimmunoblastic T cell lymphoma (AITL) description, 302 features, 303 follicular T helper cells, 34, 303 histologic patterns, nodular form, erythematous rash, 18 non-atypical lymphocytes with eosinophils, 303, 304 systemic, 303 Angiosarcoma clinical features, 572 electron microscopy, 574 epidemiology, 571, 572 immunohistochemistry and diagnosis, molecular/genetics, 574 prognosis/course, 574 H.D. Cualing et al. (eds.), Cutaneous Hematopathology, DOI / , Springer Science+Business Media New York

2 586 Annular lichenoid dermatitis clinical features, 151 diagnosis, 152 epidermotropic cells, 152, 153 genetics and molecular findings, 151 histopathology and immunophenotype, 151 hypopigmented MF, 152, 153 lymphocytes, 152 and mycosis fungoides, 152 prognosis and clinical course, Anthrax bacilli with characteristic capsule, 387, 388 clinical features, cultures and biopsies, 387 differential diagnosis, etiology and epidemiology, 386 immunostaining, 388, 389 marked edema of dermis, 387 Steiner silver stain, 387, 389 vasculitis, vascular necrosis and hemorrhage, 387, 388 Antigen presenting cell (APC), 374 AR. See Actinic reticuloid (AR) Asteroid bodies, 450, 461 Atopic dermatitis clinical features, 135 diagnosis, 136 differential diagnosis, 137, 147, 150, 160 genetics and molecular findings, 136 histopathology and immunophenotype, LCH, 165, 166 lichen simplex chronicus, 159 and mycosis fungoides, 136 pathogens and dendritic cells, 31 prognosis and clinical course, 136 and psoriasis, 26 Atypical hyperplasia, 5, 91, 93 Atypical lymphocytes actinic reticuloid, 238 basilar lining, lichen sclerosus, 149 epidermotropism, 177 formation, 183 hair follicle, 142 HIV-related dermatitis, 157 hyperchromatic/vesicular nuclei, 189, 190 KFD, 365 large, 139, 143, 300 lichenoid keratosis, 233, 234 medium-size, 175 melanoma, 544 MF, 10, 152, 153 moderately, 149 Pautrier s microabscesses, 10, 189, 190 pityriasis lichenoides chronica, 163 and prominent epidermotropism, 101 Atypical lymphocytic lobular panniculitis (ALLP), 21, 67, Atypical mycobacteriosis abundant immunostaining, 408, 409 clinical and histopathologic features, differential diagnosis, 409 epidermal ulceration, 408 humans infections, 403 immunosuppression, 405 necrosis coagulative, 408, 410 and lymphohistiocytic inflammation, 408, 409 neutrophils, monocytes, macrophages and early lesion, 408 transmission, 403 Ziehl Neelsen acid-fast stain, 408 Autoimmune lymphoproliferative syndrome (ALPS), 366, 370 B Bacterial infections anthrax, atypical (see Atypical mycobacteriosis) cat-scratch disease, definitive diagnosis, 386 mycobacterial (see Mycobacterial infections) pathogens, 386 plague (see Plague) rickettsial (see Rickettsial infections) scrub typhus, treponemal diseases, tularemia, venereal syphilis (see Venereal syphilis) Benign erythroderma, , , 225 Benign follicular mucinosis, Benign granulomatous lesions diffuse, nodular and perivascular, 119 drug-induced lymphoid and granulomatous lymphoid dyscrasia, 120 GA-like pattern, 119 pseudolymphomas, 118, 121 sarcoid-like granulomas, 120, 121 Birbeck granule CSHR, 508 FDCS, 505 ICH, 511 IDCS, 504 RD cells, 369 tennis racquet shape, 508 upper dermis, 235 Blastic plasmacytoid dendritic cell neoplasm (BPDCN) allogeneic stem cell transplantation, 503 clinical features, 493 cytogenetic analysis, 501 definition, low-level bone marrow involvement, 502 lymphadenopathy, 501 myelomonocytic disorders, 503 neoplasm, 501 plasmacytoid cytological appearance, 501, 502 radiation therapy, 503 tumor cells, 501 Index

3 Index B-lymphocyte ontogeny, 207, 208 Borrelia burgdorferi B lymphocytes, 253 CBPL, 209 cutaneous infections, 378 etiology, 209 PCMZL, 257 serum antibodies, 218 Borreliosis clinical course, 218 cytogenetics and molecular findings, 218 definition, 217 differential diagnosis, 218 features, 217 histopathology, immunophenotype, 218 BPDCN. See Blastic plasmacytoid dendritic cell neoplasm (BPDCN) C calcl. See Cutaneous anaplastic large cell lymphoma (calcl) Capillary electrophoresis (CE), 90, 91, 95, 96, 104, 283 Carcinomas clinical features, 553 diagnosis, 553, epidemiology, 553 immunohistochemistry, 553, 555 lymphoepithelioma, metastatic (see Metastatic carcinoma) molecular/genetics, 556 prognosis/course, 556, 557 catll. See Cutaneous adult T-cell leukemia/lymphoma (catll) Cat-scratch disease Afi pia felis, 394 Bartonella henselae, 394 Bartonella quintana, 394 clinical features bacillary angiomatosis, 394 diagnosis, immunostaining, 397 lichenoid lymphoplasmacytic infiltration, 396 lymphadenopathy, 394 necrosis in dermis with variable shapes, 395 palisading arrangement, 395 serologic and PCR assays, 395 skin lesions, 394 skin test, 395 Warthin Starry silver stain, 396 differential diagnosis, 397, 403, 412 human, 394 CBCL. See Cutaneous B-cell lymphomas (CBCL) CD20 inhibitor, 216, 272, 277, 293, CD52 inhibitor, 471 CE. See Capillary electrophoresis (CE) CEMH. See Cutaneous extramedullary hematopoiesis (CEMH) CHP. See Cytophagic histiocytic panniculitis (CHP) 587 Chromogenic in situ hybridization. See Immunohistochemistry Chronic lymphocytic leukemia (CLL), 120, 224, , 497 CLA. See Cutaneous lymphocyte antigen (CLA) CLH. See Cutaneous lymphoid hyperplasia (CLH) CLL. See Chronic lymphocytic leukemia (CLL) Crescentic macrophage, 362, 363, 366 CTCLs. See Cutaneous T-cell lymphomas (CTCLs) Cutaneous adult T-cell leukemia/lymphoma (catll), Cutaneous anaplastic large cell lymphoma (calcl), 194, 545, 556 Cutaneous B-cell hyperplasia, 120, Cutaneous B-cell lymphomas (CBCL) vs. CBPL, 223, 226 and CTCL, 91 drug eruptions, 215 large-cell lymphocytoma, 211 Cutaneous B-cell pseudolymphomas (CBPL) vs. CBCL, 223, 226 CD20 and CD79a+, 216 characteristics, 209 large-cell lymphocytoma, 211 polyclonal light chains, 213 Cutaneous extramedullary hematopoiesis (CEMH) child post-chemotherapy, 495 definition, infiltrative process, 496 lesions, 495 myelofibrosis, 495 myeloid malignancies and hemoglobinopathies, 493 nonhepatosplenic EMH, 495 perivascular, 495 Cutaneous gamma-delta T-cell lymphoma activation, 297 cytogenetics and molecular findings, 301 cytomorphology, 298, 300 definition, 297 differential diagnosis, epidemiology, 297 hepatosplenic, 296 in human, 297 immunophenotype, LDH, 297 pattern of infiltration CD8+ γδ lymphoma, 297, 299 mixed epidermal, mid-dermal, periadnexal and subcutaneous, 298 perivascular and nodular extensions, 298, 299 primary, 296 solitary lesions, 297 ulcerate nodules, 297 WHO-EORTC, 297 Cutaneous hematopathology ancillary tests, 89 benign erythroderma, benign follicular mucinosis, benign granulomatous lesions, clonality,

4 588 Cutaneous hematopathology ( cont.) cutaneous B-cell hyperplasia, 120, cytochemistry, 89 dermatitis, dyscrasia and prelymphoma, flow cytometry, hypopigmented interface variant, 111 image cytometry, limitation equivocal, oligoclonal/pseudoclonal, 101 oligoclones, 102 pseudoclonality and lymphocyte, 103 TCR gamma/delta gene segments, 102 tumor cells, 98 lupus profundus, MF characteristics, neoplasms, 89 parapsoriasis, PCR (see Polymerase chain reaction (PCR)) pityriasis lichenoides, 113 pseudolymphomas, solitary T-cell nodule, 122, 124 syringolymphoid hyperplasia with alopecia, Cutaneous HTLV-1 Adult T-cell leukemia/lymphoma (ATLL), Cutaneous intravascular conditions intralymphatic histiocytosis, large B-cell lymphoma, large NK-/T-cell lymphoma, Cutaneous lymphocyte antigen (CLA), 182, 183 Cutaneous lymphohematopoietic infiltrates cytometry, 8 description, 3 epidemiology, 5 germinal centers, 8 IgH/T-cell receptor gene, 8 Karyorrhexis, 9 lymphoid lesion, 7 mantle cell lymphoma, 9 melanoma evaluation, 9 monomorphic, 3 nonneoplastic and neoplastic, 3, 4 Pautrier s microabscess, 9 skin biopsy, 7 Cutaneous lymphoid hyperplasia (CLH) atypical, 94, 122 B-cell type, 5 vs. CBCL, 90 classification, 6 clonal dermatitis, 90 definition and diagnosis, 5 prominent histiocytic component, 209 pseudolymphoma, 5 secondary to foreign bodies clinical course, 216 clinical features, 215 cytogenetics and molecular findings, 216 definition, 215 differential diagnosis, Index histopathology, immunophenotype, 216 T-and B-cell pseudolymphomas, 6 Cutaneous lymphoid infiltrates CD20 inhibitors, chronic hydroxyurea, G-CSF (see Granulocyte colony-stimulating factor (G-CSF)) HFSR, interferon, RS, TNF-a inhibitors, Cutaneous lymphoid tissue CD4, CD8 and Th17 cells, 28 chemokine receptors, 28 cytokines, 26 innate immune system, 25 ligand-receptor and chemokine, 26 memory T cells, secondary immune response, serum cytokine/receptor levels, 28 skin, environment, 25 subsets, T helper cell, 28, 29 Cutaneous myelomonocytic infiltrates BPDCN, CEMH, dermal dendrocytic infiltrates, FDCS, IDCS, immunophenotypical markers, 493, 494 indeterminate dendritic cell infiltrates, LCH, lymphoblastic leukemia, 512, 513 MDS/MPD, MS, myelomonocytic skin lesions, 493 peripheral blood, 512, 513 signs, epidemiological and pattern, 493, 494 Cutaneous plasmacytoma B-cell lymphoma, 260 diffuse proliferation, 260, 261 IgG4-related skin disease, 261 tumor cells, 260, 261 Cutaneous pseudolymphoma (CPL), 208, 209, 220, 224, 225 Cutaneous T-cell lymphomas (CTCLs), 181, 182, 186, 187, 190, 191 Cutaneous T-cell pseudolymphomas (CTPL), 209, 214, 217 Cytology, 30, 166, , 194, 292, 308, 309, 327, 348, 349, 522, 532 Cytomorphology actinic reticuloid, 237 cutaneous gamma-delta T-cell lymphoma, 298, 300 extranodal NK/T-cell lymphoma-nasal type, 308, 309 FDCS, intralymphatic histiocytosis, 533 LCD, 241 lichen aureus, 235 lichenoid keratosis, 234

5 Index lymphomatoid drug reactions, 243 molecular genetic analysis, 4 mycosis fungoides, PCSM-TCL, 287, 289, 290 reactive panniculitis disorders, 329 solitary T-cell pseudolymphoma, Cytophagic histiocytic panniculitis (CHP), 21, 118, 326 D Denaturing gradient gel electrophoresis (DGGE), 95 96, 154 Dengue fever, Dermal dendrocytic infiltrates, Dermotropic reactions dermal wedge-shaped and perivascular pattern, 19 diffuse pattern, eosinophils, 18 epidermotropic (see Epidermotropic reactions) granulomatous pattern, 16 nodules, vessels, 17 plasma cells, pseudolymphomatous B-cell pattern, DGGE. See Denaturing gradient gel electrophoresis (DGGE) Diabetes granuloma annulare, 375 insipidus, 509 interferon, 478 LCH, 507, 509 Direct immunofluorescence KFD, 365 lichenoid lupus erythematosus, 158, 159 pseudodermatomyositis, 486 Dyscrasia cutaneous T-cell lymphoid, 94 erythroderma, follicular mucinosis, granulomatous, parapsoriasis, pityriasis lichenoides, 113 syringolymphoid, E EBV. See Epstein Barr virus (EBV) Embolia cutis medicamentosa, EMH. See Extramedullary hematopoiesis (EMH) Emperipolesis diagnosis, 367 engulf intact inflammatory cells, 366 intact lymphocytes, 368 neutrophils, 369 recognition, 370 S100 immunostain, 16, 17, 366, 367, 369 EORTC. See European Organization for Research and Treatment of Cancer (EORTC) Epidermotropic reactions actinic reticuloid, annular lichenoid dermatitis, atopic dermatitis, CD3 staining, 12 HIV, inflammatory vitiligo like macules, langerhans cell hyperplasia, and lichenoid dermatitis, 4, 12 drug eruptions, pattern, lichen sclerosus, lichen simplex chronicus, lupus erythematosus (see Lupus erythematosus) lymphomatoid contact dermatitis, drug eruption, lichenoid keratosis, PLC, PLF, PPD (see Lichen aureus/pigmented purpuric dermatosis (PPD)) T-cell neoplasms, 195, 196 Epidermotropism atypical lymphocytes, 177 follicular mucinosis, 180 MF/SS, 192 neoplastic lymphocytes, 178 patch/plaque lesions, 200 Pautrier s microabscess, 202 Epithelial membrane antigen (EMA) and ALK, 69 cutaneous plasmacytoma, 260 expression, 70 immunohistochemical panels, 73, 74, 83 investigation, cutaneous lymphoma/leukemia, 36 LELC, 553 MCC, 551 skin involvement, 107 systemic ALCL, 254, 356 Epstein Barr virus (EBV) cutaneous myelomonocytic infiltrates, 494 extranodal NK/T-cell lymphoma, 277, 302, 308, 310, 328, 503, 529 FDCS, 404 functions, 423 histologic features, 484 inflammatory pseudotumor-like variant, 505 intravascular large NK-/T-cell lymphoma, 529 Kikuchi-Fujimoto disease, 361, 363 LELC, 553 lymphoma, 67 microbiology, 484 negative staining, 524 oligoclonal/monoclonal, 303 and RD cells, 369 secondary oligoclonal/monoclonal, 303 in situ hybridization, 261, 264 T-cell receptor gene, 310 TCR-gamma clonality, 67

6 590 Erythroderma benign, , 192, , 225 clinical features, 166 clonal, 94 diagnosis, 168 exfoliative, 240 genetics, molecular findings and peripheral blood analysis, 167 histopathology and immunophenotype, 167 HIV, 155 prognosis and clinical course, reactive, Sézary syndrome, 142, 182, 186, 242 T-cell lymphocytosis, 4 Euroclonality consortium, 90, 91, 93 European Organization for Research and Treatment of Cancer (EORTC), 253, 261 Extramedullary hematopoiesis (EMH), , 500, 512 Extranodal NK/T-cell lymphoma-nasal type clinical behavior, 310 cytogenetics and molecular findings, cytomorphology cytological spectrum, 308, 309 medium-large atypical cells, 308, 309 polymorphous infiltrate, 308 definition, 304 differential diagnosis, epidemiology, 304 immunophenotype, oropharyngeal lesions, ulcerated mucosa, 305 pattern of infiltration angiodestructive growth pattern and occlusion, 305, 307 epidermotropism, 305, 306 ischemic necrosis, 305, 307 perivascular atypical lymphocytes, 305, 306 race predilection, 305 skin involvement, 305 F FDCS. See Follicular dendritic cell sarcoma (FDCS) Fine needle aspiration (FNA), 189 Flow cytometry, cutaneous hematopathology B lymphocytes, 104 clonal T cells, 104, 106 fresh tissue, 105 immunohistochemistry, 103 skin biopsy, 103 Vbeta probes, 104, 105 FNA. See Fine needle aspiration (FNA) Follicular dendritic cell sarcoma (FDCS) B cells, 505 CD21+, 61 clinical behavior, cytomorphology, differential diagnosis, 507 epidemiology, 505 histiocytes, 506 immunoglobulin and T-cell receptor genes, 506 immunophenotype, 506 lesions, 505 lymphadenopathy, 505, 506 ovoid cells, 505 pattern of infiltration, 505 peripheral nerve sheath tumors, 506 Follicular T helper (Tfh) cells, 30, 295, 303 Folliculotropic MF, 180, 181 Foreign body granuloma, 374, 442, 443 Forkhead box protein (FOX-P1), 62, 257, 260 Fungal infections alternariosis septate hyphae, , 466 suppurative and granulomatous inflammation, 446, 451 blastomycosis, 446, candidiasis, 446, classic histoplasmosis, 446, 457 clinical, epidemiological and histopathologic features, coccidioidomycosis, 446, cryptococcosis, 446, 455 diagnosis, 446 eumycetoma, 446, 456 hyalohyphomycosis, 446, 458 inflammatory responses, 446 multiple special stains, 446 penicilliosis, 446, 459 phaeohyphomycosis, 446, rhinosporidiosis, 446, 460 sporotrichosis, 446, 461 superficial and deep cutaneous, 446 zygomycosis, 446, 462 Index G GA. See Granuloma annulare (GA) Granulocyte colony-stimulating factor (G-CSF) cutaneous reactions, 475 neutrophilic dermatoses, 475 pyoderma gangrenosum, 477 sweet syndrome, Granuloma annulare (GA) adverse reactions, 472 description, diabetes, 375 differential diagnosis, forms, 375 fragmented collagen and abundant mucin, 375, 377 genetics and molecular findings, histology features, 472 immunophenotype, 377 interstitial, 376, 377 neoplastic conditions, 375 palisading, central necrobiosis, pathogenesis, 375, 472 perforating, 378 spindle-shaped histiocytes, 375, 376 subcutaneous, 376, 378

7 Index TNF-α inhibitor, 472 transepidermal elimination, necrobiotic collagen, 376, 378 vascular changes, 377 Granulomatous and suppurative dermatitis, 479 Granulomatous disorders, 181 Granulomatous rosacea clinical features, 225 cytogenetics and molecular findings, 225 differential diagnosis, 225 histopathology, 225 immunophenotype, 225 Granulomatous slack skin (GSS), , 184 GSS. See Granulomatous slack skin (GSS) H Hand-foot skin reaction (HFSR) alopecia, 480 chemotherapeutic agents, 482 epidermal necrosis/vesiculation, 481, 482 necrotic keratinocytes, 481 palmoplantar lesions, 481 podiatric measurement, 482 sorafenib and sunitinib, 481 VEGF, 482 Hansen disease. See Leprosy/Hansen disease HCC. See Hepatocellular carcinoma (HCC) Hematopathology. See Lymphoid reactions Hepatocellular carcinoma (HCC), 480 Herpes clinical features, 220 cytogenetics, 220 definition, differential diagnosis, 220 HHV-6, 361, 368 histopathology, 220 HSV2, 49 immunophenotype, 220 molecular findings, 220 simplex virus, 35, 69, 209, 422, 426 HFSR. See Hand-foot skin reaction (HFSR) HHV-8. See Human herpes virus 8 (HHV-8) Histiocytic necrotizing lymphadenitis, 361 Histiocytic pseudorosette, 379 HIV. See Human immunodeficiency virus (HIV) HPC. See Non-hemophagocytosis (HPC) HTLV-1 adult T-cell lymphoma/leukemia (ATLL), Human herpesvirus-6 (HHV-6), 361, 363, 368 Human herpes virus 8 (HHV-8), 255, 264, 423 Human immunodeficiency virus (HIV) acute, 423, 431 bacillary angiomatosis, 394 cat-scratch disease, 394 causes, AIDS, 422 chemotherapy, 264 clinical features, 155, 423 cutaneous acanthamebiasis, 436 dermatitis, 155, 156 diagnosis, 156 genetics and molecular findings, 155 histopathology, 423 histopathology and immunophenotype, 155 M. avium-intracellulare infection, 407 mycobacterial skin infections, 405 nuclear hyperchromasia, 157 papillary dermis, 155, 156 plasmablastic/anaplastic lymphoma, 264 prognosis and clinical course, 156 skin diseases, 423 sweat duct, 157 syphilis, 219, 414, 416 T-helper cells, 423 T. pallidum infection, 416 Hydroxyurea, Hypopigmented MF, I IDCS. See Interdigitating dendritic cell sarcoma (IDCS) Idiopathic lymphocytoma cutis clinical course, clinical features, 210 cytogenetics and molecular findings, 212 definition, 209 differential diagnosis, 213 histopathology, immunophenotype, 212 IFN. See Interferon (IFN) IgG4-related sclerosing disease, 370 IHC. See Immunohistochemistry (IHC) Image cytometry cutaneous lymphoid infiltrates, 109 immunostain enumeration, 107 Ki67-positive T cells, 107, 108 LyP, calcl and systemic ALCL, 109 Immunohistochemistry Bcl2, 32, 46 Bcl6, 32, 47 CD4, 32, 38 CD10, 32, 40 CD14, 56 CD34, 32, 42 CD56, 32, 44 CD68, 32, 44 CD117, 32, 45 CD123, CD138, 32, 45 CD279, 32, 46 CD1a, 32, 36 CD79a+ B cells and plasma cells, 32, 45 CD30+ lymphoproliferative disorders vs. reactive Processes vs. lymphoma ALK and EMA, 69 immunoprofile, 73, 74 infections, 69 LyP and ALCL, multiple biopsies,

8 592 Immunohistochemistry (cont.) CD23-positive infiltrate, 32, 41 CD8-positive T cells, 32, 39 cutaneous lymphoma/leukemia, cyclin D1, 32, 47 dermal dendrocytes, 32, 48 diagnosis, 55 diffuse follicle center vs. cutaneous diffuse large B-cell lymphoma, 62 follicular vs. folliculotropic mycosis fungoides, herpes simplex immunostain, 32, 49 interfollicular T cells, 32, 38 Ki67, 32, 49 LCA, 32, 43 lymphohematopoietic cutaneous diseases, 32 lymphomatoid drug eruption vs. MF CD4+ and CD8+ cells, 75, CD7/loss of multiple T-cell antigens, 75 delayed type hypersensitivity reaction, 74 epidermotropism, MUM1/IRF-4, 32, 48, myeloid sarcoma immature cells, 32, 43 neoplastic CD30 large cells, 32, 42 nuclear contour, 32, 37 OCT2, paler staining centers, 32, 41 panel selection, 83 pankeratin, 32, 51 pan T-cell staining, 32, 37 PAX5, 57 PD1, reactive lymphoid hyperplasia vs. low-grade B-cell lymphoma, 58 59, recognition, antigen-antibody, 55 Reed-Sternberg cell, 32, 40 S100, 32, 50 SPTCL vs. CGD-TCL, 62, 66, 69 T cells partly stained, CD7, 32, 39 TCR gamma, 58 TdT nuclear positivity, 32, 50 Immunohistochemistry (IHC) ancillary assay, 428 antibody dengue virus, 431 F. tularensis, 392 leishmaniasis, 434 Y. pestis, 390 cutaneous anthrax, 388 immunophenotype, 89 lymphoma, 401 monoclonal antibodies, 187 MxA, 330 myeloid-monocytic markers, 496 sentinel lymph node biopsy, 545 Indeterminate cell histiocytosis (ICH), 511 Indeterminate dendritic cell sarcoma, 502 Infectious diseases bacterial (see Bacterial infections) cutaneous and skin flora, 385 Index cutaneous lesions, 385 emergent and tropical pathogens, 385 fungal (see Fungal infections) medical interventions, 386 parasitic (see Parasitic infections) virus (see Viral infections) Inflammation/nonspecific dermatitis, Inflammatory pseudotumor (IPT) clinical features, 224 cytogenetics and molecular findings, 224 definition, differential diagnosis, 224 FDCS, 505, 507 histopathology, 224 IDCS, 505 immunophenotype, 224 and RDD, Inflammatory vitiligo like macules clinical features, 154 diagnosis, 154 genetics and molecular findings, 154 histopathology and immunophenotype, 154 hypopigmented patches, prognosis and clinical course, 154 Innate immune response, 25, 29 Interdigitating dendritic cell sarcoma (IDCS), Interface dermatitis characterization, 192 civatte bodies, 192 dermal-epidermal junction, 192 lymphomatoid drug reaction, 192, 193 pink colloid bodies, 192 Pityriasis lichenoides, 133 vacuolar, 13 Interferon (IFN) alopecia, 478, 480 embolia cutis medicamentosa, granulomatous and suppurative dermatitis, 479 host-derived glycoprotein, 477 local cutaneous necrotizing lesions, lupus erythematosus-like reaction, 479 psoriasis, 478 Interstitial granulomatous dermatitis. See Granuloma annulare (GA) Interstitial mycosis fungoides. See Mycosis fungoides (MF) Intralymphatic histiocytosis angioendotheliomatosis, 530 inflammation, 532 lymphatic markers, 533 lymphocytes/endothelial cells, 530 mononuclear cytomorphology, 533 papules and plaques, 532 rheumatoid arthritis, 532 skin-directed therapy, 533 synopsis, 532 Intravascular large B-cell lymphoma anthracycline-based multi-agent chemotherapy, 526 cell surface markers, 525 chromosomal abnormalities, 525

9 Index clinical forms, 523 constitutional symptoms, 522 definition, EBV, 524 HPC, 523 immunoglobulin light chain restriction, 524 immunohistochemical studies, 528 internal organs, 523, 525 livedoid erythema, 526 lung, 523, 527 lymphocytes, 523, 526 lymphovascular invasion, 527, 528 parameters, 526 skin and CNS, synopsis, 521, 522 telangiectatic erythema, 522 tumor cells, 521 Intravascular large NK-/T-cell lymphoma ALCL, 530, 531 anaplastic large-cell lymphoma, 529 blood vessels, 529 inflammatory conditions and neoplasms, 530 malignant lymphocytes, 529 overlapping immunophenotypic markers, 528 stratification, 530 synopsis, 528, 529 J Jun B proto-oncogene (JUNB), 183, 184, 196, 355 K Kikuchi-Fujimoto disease (KFD) autoimmune disorders, 361 CD68, 363, 365 crescentic macrophages, 362 cutaneous lesion, diagnosis, 361 differential diagnosis, 365 fever and cervical lymphadenopathy, 361 genetics, 364 immunophenotype, karyorrhectic debris, 362, 363 plasmacytoid monocytes, 362, 363 prognosis, skin lesions, , 366 vacuolar interface change with necrotic keratinocytes, virus, 361 Kikuchi s granulomatous dermatitis, 16 Kimura s disease, 18, 98 L Langerhans cell histiocytosis (LCH) CD1a, CD45 and CD101, 508 cutaneous, cutaneous antigen presenting cells, 507 differential diagnosis, 511 and EMH, 494 eosinophilic neoplastic cells, 368, 508 Hand-Schuller-Christian disease, 507 papules, 166 placental alkaline phosphatase, 508 self-limited disease, 509 ultrastructural finding, 508 xanthomatous component, and XG, 370 X-linked androgen receptor gene assay, 509 Langerhans cells (LC) and CD1a, 136, 370, 508 hyperplasia clinical features, 164 diagnosis, epidermotropic lymphocytes, 16 histopathology and immunophenotype, 165 nonneoplastic infiltration, 4 prognosis and clinical course, 165 MF, Large-cell transformation (LCT) clinical tumors, 180 definition, in MF (LCT-MF), 194, 356 LC. See Langerhans cells (LC) LCA. See Leukocyte common antigen (LCA) LCD. See Lymphomatoid contact dermatitis (LCD) LCH. See Langerhans cell histiocytosis (LCH) LCT. See Large-cell transformation (LCT) Leprosy/Hansen disease clinical and histopathologic features, 411 differential diagnosis, 412 etiology and epidemiology, lepromatous abundant acid-fast bacilli, 412, 413 dermal infiltrate with abundant foamy macrophages, , 411 diffuse dermal infiltration, lymphocytes and macrophages, 410, 411 Fite acid-fast stain, dermis, 412, 413 immunostaining, 412, 414 lesions, 410 treatment, 410 reactional, 411 Ridley-Jopling classification system, 410 tuberculoid, Leukemia, 4, 5, 8, 22 Leukocyte common antigen (LCA), 43, 260, 545, 551, 561, 571 Lichen aureus/pigmented purpuric dermatosis (PPD) adults and children, 195 basal vacuolar alteration and necrotic keratinocytes, 146 clinical course, 236 clinical features, 145 conventional, 235 cytomorphology, 235 description, 145 diagnosis, 147, 236 epidemiology,

10 594 Lichen aureus/pigmented purpuric dermatosis (PPD) ( cont.) etiologies, 234 genetics and molecular findings, 147, 236 histopathology and immunophenotype, 145 immunophenotype, 236 infiltration pattern, lesions, 234 lymphocytes, 145 mycosis fungoides, , 192, 195 Pautrier s microabscess, 146 and PPD, prognosis and clinical course, 147 Lichenoid drug eruptions clinical features, 144 diagnosis, 144 histopathology and immunophenotype, 144 lichen planus and mycosis fungoides, Lichenoid keratosis (LK) clinical appearance, 231 clinical course, 234 cytomorphology, 234 description, 231 diagnosis, 234 epidemiology, 231 genetics and molecular findings, 234 immunophenotype, 234 infiltration pattern, Lichen planus (LP), , Lichen sclerosus and clinical features, 148 cytologic atypia, diagnosis, epidermis, lymphocytes, genetics and molecular findings, 149 histopathology and immunophenotype, 148 hyperkeratosis, follicular plugging and epidermal acanthosis, 148 and mycosis fungoides, 150, 151 Pautrier s microabscess, 148, 149 prognosis and clinical course, 150 Lichen simplex chronicus clinical features, 159 diagnosis, 160 histopathology and immunophenotype, prognosis and clinical course, 160 LK. See Lichenoid keratosis (LK) Local cutaneous necrotizing lesions, LP. See Lichen planus (LP) Lupus band, 365 Lupus erythematosus (LE) and clinical features, cutaneous, 365 diagnosis, 159 discoid, 102 follicular mucinosis, 74 genetics, molecular findings and serologic studies, 158 histopathology and immunophenotype, 158, 579 lichenoid, 158, 159 Index and liver disease, 145 lymphocutaneous injection, 477, 478 panniculitis, 67 prognosis and clinical course, 158 reaction, 479 skin lesions, 366 SPTCL, 323, 328, 329 systemic, 365, 478 tumor necrosis factor-α (TNF-α), 472 Lupus pernio, 371, 374 Lupus profundus ALLP, 117, 119 bean-bag, 118, 120 CHP, 118 fat lobules and mucin deposition, 116, 118 subcutaneous lymphoid dyscrasia, 116 Lymph nodes, MF/SS core biopsy/fna, 189 MF patients, 187, 188 NCI grading system, 187 occult tumor cells, 187, 189 Vbeta8, 187 Lymphocytic infiltration clinical course, cytogenetics and molecular findings, 224 definition, 224 differential diagnosis, 225 histopathology, 224 immunophenotype, 224 Lymphoid reactions angiolymphoid hyperplasia, 20 dermal wedge-shaped and perivascular pattern, 19 diffuse pattern, 19 germinal centers, 15 histiocytes and inflammatory elements, 14 intravascular pattern, Kikuchi s granulomatous dermatitis, 16 lichenoid pattern, 13 nodular T-cell pseudolymphoma, 17 nodules, vessels, 17 plasma cells, 18 poikilodermatous MF, breast skin, sarcoidosis, 16 scant perivascular lymphoid infiltrates, 18 single-file pattern, 22 subcutaneous pattern, 21 vacuolar change, 13 vessels and eosinophils, 18 Lymphoma atypical lymphocytic infiltrates, 96 B-cell, 217 cutaneous marginal zone, 258 Hodgkin, 57 immunohistochemistry (see Immunohistochemistry) malignant, 113 mantle cell, 9 T-cell, 21 Lymphomatoid contact dermatitis (LCD) clinical course, clinical features, 136

11 Index cytomorphology, 241 description, 240 diagnosis, , 242 epidemiology, 240 genetics and molecular findings, 241 histopathology and immunophenotype, 136 immunophenotype, 241 infiltration pattern, 240, 241 lesions, 240 prognosis and clinical course, 136 Lymphomatoid drug eruption clinical features, 141 diagnosis, 142 genetics and molecular findings, 142 histopathology and immunophenotype, 142 and mycosis fungoides, 142, 143 Pautrier s microabscess and follicular mucinosis, induced pseudo B-cell lymphoma clinical course, 214 clinical features, 214 cytogenetics and molecular findings, 214 definition, differential diagnosis, 215 histopathology, 214 immunophenotype, 214 reactions clinical appearance, 242 clinical course, cytomorphology, 243 description, 242 diagnosis, 244 epidemiology, 242 genetics and molecular findings, 243 immunophenotype, 243 infiltration pattern, Lymphomatoid lichenoid keratosis basal vacuolar alteration, 138, 140 clinical features, 138 diagnosis, 141 epidermis, 138, 139 genetics and molecular findings, 141 immunophenotype, 140 infiltrate, 138, 140 lymphocytes, 138, 139 and mycosis fungoides, 141 prognosis and clinical course, 141 Lymphomatoid papulosis (LyP) associated with 6p25.3 rearrangement, clinical features, 337 definition, 337 differential diagnosis C-ALCL, 351, 352CD8 and CD56 expression, 351 CD30+ pseudolymphomatous infiltrates, 348 concomitant granulomatous inflammation, 351, 352 histologic types, 348, 349 melanoma markers, metastatic melanoma, 348 myxoid ALCL, 351 neutrophils and eosinophils, 351 PLC, 349, PLEVA, PVB19, 350 scabies and pityriasis lichenoides, 348 eosinophils, 339, 340 epidemiology, 337 features, 339, 341 genetics adult female, 343 CD30+ cells, 347 chromosomal rearrangements, 6p25.3, 343, 346 clonality, DUSP22 allele, 346 punch biopsies, 346 T-cell leukemia and non-hodgkin lymphomas, 343 TCR gene rearrangements, 346 young female, 343 histology, early lesion, 339 immunophenotype atypical cells expression, CLA, 342 CD30 antigen, 342, 346 Hodgkin lymphoma-associated antigens, 342 TIA-1 representing cytotoxic proteins, 342, 346 lesions, 337, 338 macrophages, 339 neutrophils and ulceration, 339, 340 and nonlymphoid malignancies, pagetoid reticulosis, 341, 344 6p25.3 rearrangement, 342, 345 Reed-Sternberg-like cells, 340, 342 type A, 339, 341 type B, 195, 197, 340, 343 type C, 340, 344 type D and E, 341, 344 Lymphomatosis, 521 LyP. See Lymphomatoid papulosis (LyP) M MCC. See Merkel cell carcinoma (MCC) MDS/MPD. See Myelodysplastic syndrome/ myeloproliferative disorders (MDS/MPD) Melanoma amelanotic, clinical features, 540 cytologic preparations, 544 diagnosis, 540 electron microscopy, 547 epidemiology, 540 halo nevus, Hodgkin lymphoma, 543 immunohistochemistry, 546, 547 lymphocytes, 543 metastatic, molecular/genetics, prognosis/course, 548 small cell melanoma,

12 596 Index Merkel cell carcinoma (MCC) clinical features, 549 diagnosis, electron microscopy, 552 epidemiology, 549 immunohistochemistry, molecular/genetics, 552 prognosis/course, Metastatic carcinoma breast adenocarcinoma, 560, 561 dermis, 560 GCDFP15, 560 leukemia cutis, 561 myeloperoxidase, 561 neoplasms, 560 clinical features, 558 epidemiology, 557, 558 immunohistochemistry, MF. See Mycosis fungoides (MF) Monkeypox clinical features, 425 cytopathic effect, 425, 427 differential diagnosis, 426 etiology and epidemiology, 425 marked acantholysis and full-thickness necrosis epidermis, 425, 426 mildly acantholytic epidermis, early stage, 425, 426 orthopoxvirus antigens, 426, 427 PCR assays, 425 spectrum of changes, skin lesion, 425 MS. See Myeloid sarcoma (MS) Mycobacterial infections atypical, classes, 402 leprosy (see Leprosy/Hansen disease) nonvenereal treponematoses children, 422 Pinta, T. carateum, transmission, 422 Yaws, TB (see Tuberculosis (TB)) transmission, 403 treponemal diseases, venereal syphilis (see Venereal syphilis) Mycobacterium marinum, 378, 408, 409 Mycosis fungoides (MF) actinic reticuloid, 161 and atopic dermatitis, 136 c-alcl, 270 CTCL, 269 cytomorphology, definition, 175 dermatitis, 137 differential diagnosis, 191, 192 epidemiology, 175 epidermotropism, 135 eruption, lymphomatoid drug ( see Lymphomatoid drug) folliculotropic, 180, 181 granulomatous, , 184 GSS, , 184 HIV, 158 hypopigmented, 153, independent prognostic factors, 189, 190 infiltration pattern algorithm, diagnosis, 177, 179 epidermis, 175 epidermotropism, 175, 177 LC, lymphocytes, 176, 178 Pautrier s microabscess, 176, 177 inflammatory vitiligo-like macules, 154 LCH, lesions, 175, 176 lichen sclerosus, 151 lichen simplex chronicus, 160 lymphomatoid lichenoid keratosis, 141 and non-mf, 270 pagetoid reticulosis, 182, 185 papular, 180 pigmented purpuric dermatosis, 147 poikilodermatous, 180, 182 PPD, 147 transformed (see Transformed mycosis fungoides (MF)) WHO-EORTC data, 270 Myelodysplastic syndrome /myeloproliferative disorders (MDS/MPD), Myeloid sarcoma (MS), N Naked granuloma, 371, 372 NB. See Neuroblastoma (NB) Necrobiosis central, 375, 376 granuloma annulare, 375 lipoidica, 378 and mucin deposition, 472 palisaded histiocytic infiltration, 221 Neoplastic epidermotropic diseases. See Mycosis fungoides (MF) Neuroblastoma (NB) clinical features, 575 epidemiology, 574, 575 immunohistochemistry and diagnosis, molecular/genetics, 576 prognosis/course, Nodular B-lymphocyte reactive patterns B-lymphocyte ontogeny, 207, 208 epidemiology, 209 etiology, 209 nomenclature, reaction pattern/s, Non-hemophagocytosis (HPC), 523 Nucleophosmin (NPM),

13 Index O Osteosarcoma (OS) clinical features, 569, 570 epidemiology, 569 immunohistochemistry and diagnosis, molecular/genetics, 571 prognosis/course, 571 P Pagetoid reticulosis, 182, 185 Paired box gene 5 (PAX5), 57 Palisaded and neutrophilic granulomatous dermatitis Parapoxvirus clinical features, differential diagnosis, 428 epidermis, 428 etiology and epidemiology, immunostaining, 428, 429 keratinocytes, 428, 429 Parapsoriasis, Parasitic infections helminthic diseases, protozoal cutaneous lesions, 432 free-living amebae, leishmaniasis, Parvovirus B19 (PVB19), Pautrier s microabscess, , 181, 183, 185, , 194, 202 PBL. See Peripheral blood lymphocytes (PBL) PCFCL. See Primary cutaneous follicle center lymphoma (PCFCL) PCGD-TCL. See Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) PCLBCL. See Primary cutaneous large B-cell lymphoma, leg type (PCLBCL) PCMZLs. See Primary cutaneous marginal zone B-cell lymphomas (PCMZLs) PCR. See Polymerase chain reaction (PCR) Peripheral blood lymphocytes (PBL), 194, 195 Peripheral T-cell lymphomas (PTCLs) CTCL, cutaneous, 269 diagnosis, 269 MF (see Mycosis fungoides (MF)) Persistent nodular arthropod-bite reaction and nodular scabies clinical course, 223 clinical features, 222 cytogenetics and molecular findings, 222 definition, 222 differential diagnosis, 223 histopathology, 222, 223 immunophenotype, 222 Pityriasis lichenoides chronica (PLC) clinical features, 162 cytotoxic variant MF, 113 diagnosis, 164 genetics and molecular findings, 163 histopathology and immunophenotype, 162 intraepidermal involvement, 162, 163 LyP type B, 195, 197 and PLEVA, 192, 349, prognosis and clinical course, 164 Pityriasis lichenoides et varioliformis acuta (PLEVA), 192, Plague clinical cutaneous manifestations, 390 differential diagnosis, 390 histopathologic features immunostaining, 390, 392 purpuric and petechial lesions, 390 septicemic, abundant gram-negative bacilli, 390, 391 vasculitis and vascular necrosis, 390, 391 incubation period, 390 necrotic lesions, 390 primary pulmonary infection, 390 serologic and PCR assay, 390 transmission, 390 Yersinia pestis, 389 zoonotic infection, 390 Plasmablastic lymphoma (PBL) clinical features, 264 cytogenetics and molecular findings, 264 histopathology, 264 and HIV, 264 immunophenotype, 264 Plasmacytoid monocytes, 362, 363, 365 PLC. See Pityriasis lichenoides chronica (PLC) PLEVA. See Pityriasis lichenoides et varioliformis acuta (PLEVA) PLF. See Pseudolymphomatous folliculitis (PLF) Poikilodermatous MF, 180, 182 Polymerase chain reaction (PCR) B-lymphocyte ontogeny, 285 cat-scratch disease, 395 CE, 95 clonal dermatitis, 91, 191, 208 cutaneous lymphomas and hyperplasias, 90 DGGE, EuroClonality consortium, 91, 93 lymphocytes, 91, 92 monkeypox, 425 plague, 390 Sézary syndrome, 185 spotted fever group rickettsioses, 398 T and B receptor gene rearrangement assays, 90 TCR and BCR clonality assay, 90 tuberculosis, 403 Poxvirus infections double-stranded DNA, 424 eradication, 425 humans, 424 monkeypox, parapoxvirus, transmission, 425 PPD. See Lichen aureus/pigmented purpuric dermatosis (PPD) 597

14 598 Index Primary cutaneous ALCL clinical features, cytoplasmic staining, ALK, differential diagnosis large cell transformation of MF (LCT-MF), 356 neutrophil-rich ALCL, 357 secondary skin lesions, 356 genetics, grenz zone, 353, 354 immunophenotype, inflammatory cells, 353 LyP, 353 neutrophils, 353, 354 sheets of, 353 ulcerated lesion and facial tumor, 352 Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, Primary cutaneous follicle center lymphoma (PCFCL) bone marrow examination, 255 cytogenetics and molecular findings, 257 germinal center cells, 254 HHV-8, 255 histopathology, 255, 256 immunophenotype, neoplastic cells, 62 reactive lymphoid hyperplasia, 58 Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) adults, 326 cytotoxic proteins, granzyme B, 328, 331 detection, TCR clonality, 328 differential diagnosis, 328 epidermal and dermal, 326, 329 interface changes, 326, 329 neoplastic epidermotropic diseases, nuclear pleomorphism, 326, 330 radiation and multi-agent chemotherapy, 326 TCR-δ, 326, 330 ulcerating lesions, 326, 328 Primary cutaneous large B-cell lymphoma, leg type (PCLBCL) clinical features, 262 cytogenetics and molecular findings, 263 and EORTC, 261 histopathology, 262 immunophenotype, 262, 263 and PCFCL, 62 Primary cutaneous marginal zone B-cell lymphomas (PCMZLs) bone marrow, 257 cytogenetics and molecular features, 260 fluoxetine therapy, 258 histopathology, 258, 259 immunocytomas, 257 immunophenotype, and PCFCL, 58, 264 tumor cells, 257 Primary cutaneous small-to medium-sized T-cell lymphoma (PCSM-TCL) CD8 + tumor categories, 285 clinical behavior, cytomorphology pleomorphic composition, 287, 289, 290 reactive small lymphocytes, 287, 289 definition, diagnosis, 285, 286, 295 epidemiology, 286 etiology, 292 immunophenotype, indolent nodular pleomorphic, 295 pattern of infiltration dense nodular involvement, 287 minimal/absent epidermotropism, 287, 288 upper portions of subcutaneous tissue, 287, 288 pleomorphic, 286 predilection, ear, 286 relationship, 295, 296 solitary reddish nodule, face, 286 T-cell pseudolymphoma CD68+ histiocytes, 293, 294 CD8 T and CD20 B cells, 293 eosinophils/plasma cells, 293, 294 histiocytes and small granulomas, 293, 295 low Ki67, 293, 294 mimic, 293 nodular pattern, TCR gene rearrangement, 291 unspecified, 295 WHO-EORTC classification, 285 Primary cutaneous T-cell/histiocyte-rich B-cell lymphoma, Programmed cell death protein 1 (PD1), Pseudo B-cell lymphoma secondary to infections borreliosis, herpes, syphilis, Pseudolymphomas band-like and nodular/ diffuse, 98, B-and T-cell, 6 cutaneous, 4, 14, 92, 94 discoid lupus, 98, 102 drug list, 97, 109 folliculitis, 14 gene rearrangement assay, 95, 96 GeneScan, 94 heteroduplex assay, 95 hypersensitivity reaction, lymphomatoid, 98 immunostains, 95, 97 lichenoid benign lymphomatoid keratosis, 98, 101 pattern, 98, 99 lichen planus, 98, 99 lichen sclerosus, 98, 100 lymphomatoid folliculitis, 98, 102 mimic cutaneous lymphoma, 96 oligoclonal pattern, 94 prelymphomatous infiltrate, 94, 98

15 Index skin, 5 T-/B-cell infiltrates, 94 vaccination, Pseudolymphomatous folliculitis (PLF) folliculotropic MF, 14 germinal centers, 15 hair follicles, 14 Psoriasis, 194 Pyoderma gangrenosum, 477 R RCC. See Renal cell carcinoma (RCC) Reactive cutaneous lymphoid hyperplasia, 209 Reactive lymphoid hyperplasia vs. low-grade B-cell lymphoma immunoprofile, neoplastic cells, marginal zone, PCFCL, 59, 61 PCMZL, 58 Reactive panniculitis disorders erythema nodosum (EN), 332, 333 infections, LEP and ILLP characteristics, 330 cytomorphology, 329 dense lymphocytic infiltrate without atypia, 329, 332 differential diagnosis, interface dermatitis, 329 nodular lymphocytic infiltration, 328, 331 Reed Sternberg cells, 544, 545 Renal cell carcinoma (RCC), , 558 Rhabdomyosarcoma (RMS) clinical features, 565, 566 epidemiology, 565 immunohistochemistry and diagnosis, 568, 569 molecular/genetics, 568 ovoid nuclei, 566 pleomorphic, 566, 568 prognosis/course, 569 strap cells, 566 tumor cells, 566, 567 Richter s syndrome (RS) alemtuzumab therapy, 484 bone marrow biopsy, 484 CLL, 483 EBV, 484 natural killer lymphocytes, 485 secondary lymphoma, 484 Rickettsial infections obligate intracellular parasites, 397 skin lesions, 397 spotted fever group, RMS. See Rhabdomyosarcoma (RMS) Rosai-Dorfman disease (RDD) clinical features, 366 cutaneous, differential diagnosis, extranodal manifestation, 366 Fas-encoding gene, 370 histopathology cutaneous lesions, 368 dense lymphocytic infiltrate, 367, 368 emperipolesis, Human herpesvirus-6, langerhans cell histiocytosis, , 370 nodular/diffuse infiltrate, parvovirus B19 and EBV, 369 subcutaneous fat, 367 IgG4-related sclerosing disease, 370 massive lymphadenopathy, 365 polyclonality, 370 prognosis, 370 relationship, 370 S100 immunostain, 369 sinus histiocytosis, 365 RS. See Richter s syndrome (RS) 599 S SALT. See Skin-associated lymphoid tissue (SALT) Sarcoidosis APC, 374 cytokine genes, 374 differential diagnosis, 374 familial clustering, 374 features, 371 granulomas epithelioid, 16, 181 foreign body-type giant cells, 372, 373 multisystem disorder, 371 naked, 371, 372 periadnexal and/or perivascular distribution, 372 rounded, 371, 372 tattoo granules, 373 HLAs, 374 immunophenotype, 374 lymphoma syndrome, 371 malignancies, 371 pathogenesis, 371 polarizable foreign bodies, polyclonal, 374 prognosis, 374 scar and verrucous, 372 syringotropic and verrucous, 371 Sarcomas clinical features, 564 epidemiology, 564 immunohistochemistry and diagnosis, 564, 565 leukemia, 564 molecular/genetics, 564, 565 prognosis/course, 565 small round blue cell, 564 Scar sarcoidosis, 372 scd30. See Serum soluble CD30 (scd30) Scrub typhus clinical features, 401 differential diagnosis, 401 eschar lesion, 401, 402

16 600 Scrub typhus ( cont.) etiology and epidemiology, 401 immunohistochemistry, 401 vasculitis, 401, 402 Serum soluble CD30 (scd30), 189 Sézary syndrome (SS) and ALCL, 194 benign erythroderma, blood, 189 cutaneous lesions, cytogenetics and molecular findings, description, 182, 186 differential diagnosis, 191, 192 epidermotropic T-cell neoplasms, 195, 196 immunophenotype, , 187 inflammation/nonspecific dermatitis, interface dermatitis, laboratory prognostic biomarkers, lichen aureus, 195 lymph nodes, 187, 189 lymphomatoid papulosis associated with 6p25.3 rearrangement, LyP type B, 195, 197 and PCGD-TCL, primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, psoriasis, 194 skin, Sinus histiocytosis with massive lymphadenopathy, 4, 365 Skin biopsy, 6 7, 476 breast, cutaneous hematopathology, 103 cytology, 30 dermis, directed therapy, 533 epidermis, 28 epithelioid histiocytes and granulomas, 31 HFSR (see Hand-foot skin reaction (HFSR)) histology chemokines, 11 dermis, 12 epidermis, 9 10 hypopigmented MF, 11 intercellular edema/spongiosis, 12 keratinocytes, langerhans cells (LC), 11 mycosis fungoides basal layer, 10 Pautrier s/darier s nest, 10 subcutaneous tissue, IgG4-related skin disease, 261 immunoperoxidase stain, 183, 187 intravascular large B-cell lymphoma, 522, 523, 524 involvement, EMA, 107 lesions, , 366, 394, 397, 422 monkeypox, 425 myelomonocytic, 493 NK/T-cell lymphoma, nasal type, 305 Index NK T cells and gamma-delta T cells, 31 pathogens and dendritic cells, 31 primary cutaneous ALCL, 356 pseudolymphomas, 5 rating, SALT, 28 Sézary syndrome (SS), subcutaneous tissue/hypodermis, 32 T-cell reaction, 31 test, 395 Th17 and MF, 31 Skin-associated lymphoid tissue (SALT), 28 Small cell lung cancer CK7, 561 molecular/genetics, 564 neuroendocrine tumors, 561, 562 prognosis/course, 564 tumor nuclei, 561, 562 Solitary T-cell pseudolymphoma clinical appearance, 244 clinical course, 246 cytomorphology, description, 244 diagnosis, epidemiology, 244 genetics and molecular findings, 245 immunophenotype, 245 infiltration pattern, Spindle cell B-cell lymphoma, 256 Spotted fever group rickettsioses diagnosis, RMSF, 398 differential diagnosis, 400 etiology and epidemiology, histopathology endothelial swelling, 398, 399 erythrocyte extravasation, 398, 399 Eschar formation, immunostaining, 400, 401 lymphohistiocytic perivascular infiltration, 398 microthrombi and vessel wall necrosis, 399, 400 vascular damage with leukocytoclastic vasculitis, 399 PCR testing, 398 vasculotropic, 398 SS. See Sézary syndrome (SS) Subcutaneous granuloma annulare, 376, 379 Subcutaneous lymphoproliferative disorders PCGD-TCL, 326, 328 reactive panniculitis, SPTCL, Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) CD30+ tumor cells, calcl, 326, 328 dense lymphocytic infiltration, 324, 325 diagnosis, differential diagnostic features, 326, 327 epidemiology, 323 expression, cytotoxic molecules, 324, 325 fat necrosis, 324, 325 genetics, 324, 326

17 Index hemophagocytic syndrome, 323, 324 immunophenotype, 324 lower extremities, 323, 324 and lupus erythematosus profundus, 323 systemic B symptoms, 323 Sweet syndrome apoptosis, 476 cutaneous lesions, 476 drug-induce, 475 hematologic malignancies, 475 myeloid disorder, 477 neutrophilia, 476 skin biopsy, 476 Syphilis clinical features, 219 cytogenetics, 219 definition, differential diagnosis, 219 histopathology, 219 immunophenotype, 219 molecular findings, 219 venereal (see Venereal syphilis) Syringolymphoid hyperplasia with alopecia, Syringotropic sarcoidosis, 371 Systemic lupus erythematosus CD20 inhibitors, 483 KFD, 361, 365 lichenoid, 157 lymphocutaneous injection site reactions, 478 SPTCL, 323 T TB. See Tuberculosis (TB) T-cell clonality, 89, 101, 105, 114, 118, 147, 151, 152, 154, 161, 191, 330, 347 T-cell receptor gene rearrangement (TCRGR), 236, 248 TCRGR. See T-cell receptor gene rearrangement (TCRGR) Th1, 28, 29, 31 Th2, 28, 31 The multiple myeloma oncogene 1 (MUM1)/interferon regulator factor 4 (IRF4) gene encodes, TOX and PDCD1, 185 Transformed mycosis fungoides (MF) Bcl2 proteins levels, 270 expression, activation markers, 270 follicle helper T cell, pathogenesis, 271 PTCL-u admixed histiocytes, 280 CD30 expression, cell behaviour, cerebriform/atypical dysplastic cell, 280 cytogenetics and molecular findings, 283 deep subcutaneous tissue, 280, 282 definition, 278 differential diagnosis, epidemiology, histologic large cell, nuclear irregularity, pain, 279 pattern of infiltration, 279, 280 progression, 278 Reed-Sternberg cells, 280, 281 T-cell antigens, , 283 T3 stage, 279 tumor, 278 Translocation (2;5)(p23;q35), 69, 354, 356 Tuberculosis (TB) acid-fast bacilli, 403, 407 caseating granulomas, 403 clinical and histopathologic features, 403, 404 cultures and acid-fast stains, 403 differential diagnosis, 403 diffuse mixed inflammatory infiltrate in dermis, 403, 404 lungs, 403 Mycobacterium, 403 noncaseating granuloma, 403, 405 papulonecrotic, 403, 406 PCR testing, 403 scrofuloderma, 403, 405 vaccination, BCG, 403 verrucosa cutis, 403, 406 Tularemia causes, clinical features, 392 differential diagnosis, 393 etiology and epidemiology, F. tularensis palaearctica (type B), 392 F. tularensis tularensis (type A), histopathology immunostaining, 392, 394 subcutaneous lymphadenitis, 392, 393 suppurative granuloma formation, 392, 393 Tumor mycosis fungoides, 178, 191, 194, 278, 283, 285, 301 Tumor necrosis factor-α (TNF-α) antibody inhibitors, 471 antitumor necrosis factor therapy, 471 granuloma annulare/interstitial granulomatous dermatitis, proinflammatory cytokine, 471 urticaria and cellulitis, U Unspecified peripheral T-cell lymphoma (PTCL-u) aberrant T antigens, 272, 276 CD8-positive, 272, 276 clinical behavior, 272 clustered pustular plaque/nodule, 272 coexpression, CD20, 272, 277 cytogenetics and molecular findings, 272 definition, 271 differential diagnosis, 274 epidemiology, epidermotropism, 272, 274 immunophenotype, 272 low-power histologic patterns, 272, 273