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TitleSkin Lymphoma the Illustrated Guide Cerroni 5 ed 2020
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Table of Contents
                            Skin Lymphoma The Illustrated Guide, Fifth Edition
Half Title
Title Page
Copyright
Dedication
Contents
List of teaching cases
Preface
Acknowledgments
Chapter 1 Introduction
	Classification of cutaneous lymphomas
	Examination of patients
	Staging investigations
	Surgical techniques
	Histopathology, immunophenotype, and molecular genetics
	Other methods used in the study of cutaneous lymphoid infiltrates
	Other genetic investigations
	Lymphoma microenvironment and lymphoma‐associated microorganisms
	Pseudomalignancy, premalignancy, and early malignancy
	References
Chapter 2 The “parapsoriases”: a riddle, wrapped in a mystery, inside an enigma
	References
Chapter 3 Mycosis fungoides
	Onset of mycosis fungoides or exacerbation of undiagnosed disease under treatment with immunomodulatory agents
	Clinical features
	Extracutaneous involvement
	Association with other diseases
	Histopathology, immunophenotype, and molecular genetics
	Histopathologic differential diagnosis from inflammatory conditions
	Clinical and histopathologic variants
	Prognosis
	References
Chapter 4 Seary syndrome
	Onset/progression under systemic therapies for benign inflammatory dermatoses
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	References
Chapter 5 Primary cutaneous CD30+ lymphoproliferative disorders
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Clinicopathologic differential diagnosis
	Treatment
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	References
Chapter 6 Subcutaneous panniculitis‐like T‐cell lymphoma
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Differential diagnosis with other cutaneous NK/T‐cell lymphomas with prominent involvement of the subcutaneous tissue
	Differential diagnosis with lupus panniculitis
	Treatment
	Prognosis
	References
Chapter 7 Aggressive cutaneous cytotoxic lymphomas
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	References
Chapter 8 Cutaneous peripheral T‐cell lymphoma, not otherwise specified (NOS)
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	References
Chapter 9 Cutaneous CD4+ small/medium T‐cell lymphoproliferative disorder
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	Final considerations
	References
Chapter 10 Acral CD8+ cutaneous T‐cell lymphoma
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	References
Chapter 11 Cutaneous adult T‐cell leukemia/lymphoma
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 12 Other cutaneous NK/T‐cell lymphomas
	Clinical features, histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features, histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 13 Cutaneous follicle center lymphoma
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Differential diagnosis of cutaneous follicle center lymphoma, diffuse type, from cutaneous diffuse large B‐cell lymphoma, leg t
	Treatment
	Prognosis
	References
Chapter 14 Cutaneous marginal zone lymphoma (cutaneous MALT lymphoma) and variants
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	References
Chapter 15 Cutaneous diffuse large B‐cell lymphoma, leg type
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment
	Prognosis
	References
Chapter 16 Intravascular large cell lymphomas
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Intravascular large B‐cell lymphoma colonizing hemangiomas
	Treatment and prognosis
	References
Chapter 17 Other cutaneous B‐cell lymphomas
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 18 Cutaneous lymphomas in immunosuppressed individuals
	CUTANEOUS POSTTRANSPLANT LYMPHOPROLIFERATIVE DISORDERS
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	CUTANEOUS LYMPHOMAS IN HIV‐INFECTED INDIVIDUALS
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	CUTANEOUS LYMPHOPROLIFERATIVE DISORDERS IN OTHER IATROGENIC AND NON‐IATROGENIC IMMUNE DEFICIENCIES
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 19 Cutaneous manifestations of B‐cell chronic lymphocytic leukemia
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	PROGRESSION TO DIFFUSE LARGE B‐CELL LYMPHOMA (RICHTER SYNDROME)
	References
Chapter 20 Cutaneous manifestations of myelogenous leukemia
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Immunophenotype
	Treatment and prognosis
	References
Chapter 21 Blastic plasmacytoid dendritic cell neoplasm
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Immunophenotype
	Treatment
	Prognosis
	MATURE PLASMACYTOID DENDRITIC CELL PROLIFERATIONS ASSOCIATED WITH MYELOID NEOPLASMS
	References
Chapter 22 Cutaneous manifestations of other leukemias
	SPECIFIC SKIN MANIFESTATIONS OF OTHER TYPES OF LEUKEMIA
	References
Chapter 23 Cutaneous lymphoblastic lymphomas
	CUTANEOUS B‐LYMPHOBLASTIC LYMPHOMA
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Differential diagnosis
	Treatment and prognosis
	CUTANEOUS T‐LYMPHOBLASTIC LYMPHOMA
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 24 Cutaneous manifestations of Hodgkin lymphoma
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 25 Cutaneous composite lymphomas
	Clinical features
	Histopathology, immunophenotype, and molecular genetics
	Treatment and prognosis
	References
Chapter 26 Nonneoplastic cutaneous signs of systemic lymphomas
	References
Chapter 27 Cutaneous lymphomas in children and adolescents
	References
Chapter 28 Pseudolymphomas of the skin
	Lupus tumidus and lymphocytic infiltration of the skin (Jessner–Kanof)
	Lupus panniculitis
	Other pseudolymphomas associated with cutaneous lupus erythematosus
	References
Chapter 29 The cutaneous “atypical lymphoid proliferation”
	References
Index
                        
Document Text Contents
Page 2

Skin Lymphoma

Page 292

(a) (b) (c)

Figure 14.12 Cutaneous marginal zone lymphoma. Morphologic variations of the conventional variant. (a) Marginal zone cells (“centrocyte‐like”) with
abundant cytoplasm admixed with plasma cells, small lymphocytes, and eosinophils. (b) “Monocytoid” cells, some with blastoid morphology. (c)
Lymphoplasmacytoid cells and plasma cells, admixed with some blastoid cells.

(a)

(c)(b)

Figure 14.13 Cutaneous marginal zone lymphoma, conventional variant. (a) “Top‐heavy” infiltrate with the characteristic pale and dark areas typical of
this variant of cutaneous marginal zone lymphoma. (b) The neoplastic population consists predominantly of marginal zone cells. (c) Reactive germinal
centers are present in most cases of cutaneous marginal zone lymphoma.

Page 293

CHAPTER 14 Cutaneous marginal zone lymphoma (cutaneous MALT lymphoma) and variants 283

It is possible that most of the non‐class‐switched cases fall
into  the group of cutaneous marginal zone lymphoma with
lymphoplasmacytic differentiation (the old “cutaneous immu-
nocytoma”), but IgM positivity is rare, and there are no detailed
data about its frequency in different variants.

Clusters of CD123+ plasmacytoid dendritic cells have been
reported in some cases of cutaneous marginal zone lymphoma,
and a pathogenetic role of these cells has been proposed [65].
In my experience and in that of others [64], these clusters
are  always small and are unrelated to the neoplastic cells
(Fig. 14.20). In my opinion, plasmacytoid dendritic cells do not
play any role in the disease. PD‐1+ reactive T lymphocytes have
been observed more often in cutaneous follicle center lym-
phoma than in cutaneous marginal zone lymphoma (neoplastic
cells are negative with this marker) [66], but in my experience
do not play a role in the differential diagnosis between the two
diseases.

Molecular genetics
A monoclonal rearrangement of the Ig genes can be observed in
approximately 50–60% of cases. The t(11,18)(q21;q21), detected
mostly in gastric and lung tumors, is almost never present in
cutaneous cases  [2, 36, 67, 68]. A specific interchromosomal
14;18 translocation involving IGH and MALT1 has been
described in a subset of cutaneous marginal zone lymphomas as
well as of marginal zone lymphomas of other organs including
the liver, ocular adnexa, and salivary glands [68]. It seems that
rare cases of cutaneous marginal zone lymphoma may harbor a
conventional t(14,18) involving IGH and BCL2 as well [69]. A
t(3,14)(p14;q32) involving IGH and FOXP1 has been detected
in a subset of MALT lymphomas arising at different sites,
including 2/20 cutaneous cases tested [70]. Cases showing a
trisomy 3 may be characterized by upregulation of FOXP1 [70].
Trisomy 18 is relatively common in intestinal, ocular adnexal,
and salivary gland tumors, but very rare in cutaneous cases.

(a) (b)

(c) (d)

Figure 14.14 Cutaneous marginal zone lymphoma, conventional variant. Staining for (a) CD3 and (b) CD20 shows many reactive lymphocytes, admixed
with (c) CD138+ neoplastic plasma cells showing (d) monoclonal expression of the immunoglobulin light chain κ.

Page 583

Skin Lymphoma The Illustrated Guide, Fifth Edition


Index 583

spongiosis
aggressiveepidermotropic CD8+ cytotoxic

T‐cell lymphoma, 198
atopic dermatitis, 68, 495
hydroavacciniforme‐like

lymphoproliferative disorder, 247
lymphomatoid contact dermatitis, 479
mycosisfungoides, 16, 18, 36, 38, 39, 41,

77, 80
persistent arthropod bite reactions, 503
Sézary syndrome, 120, 497

squamous cell carcinoma
lymphoepithelial‐like (see

lymphoepithelial‐like carcinoma)
pseudocarcinomatous hyperplasia in

anaplastic large cell lymphoma, 156
“squared” rete ridges, 81
staging investigations

anaplastic large cell lymphoma, 136, 150,
172, 467

atypical lymphoid proliferations, 187,
565–567

B‐cell lymphomas, 4
diffuse large B‐cell lymphoma leg‐type, 253
folliclecenter lymphoma, 255
lymphomatoidpapulosis, 136
marginal zone lymphoma, 4, 278
mycosisfungoides, 4, 7, 24, 201
peripheral T‐cell lymphoma NOS, 211
Sézary syndrome, 122

“starry sky” pattern
B‐lymphoblastic lymphoma, 337,

424, 429
Burkitt lymphoma, 339, 340
diffuse large B‐cell lymphoma leg type, 519
lymphoblastic lymphomas, 424
lymphocytoma cutis, 519, 525
myelogenousleukemia, 393
T‐lymphoblastic lymphoma, 424, 430

stem cell transplantation
adult T‐cell lymphoma/leukemia, 187
blasticplasmacytoid dendritic cell

neoplasm, 359
B‐lymphoblastic lymphoma, 429
extranodal NK/T‐cell lymphoma, 421
intravascular large cell lymphoma, 319
lymphomatoidgranulomatosis, 341
mantle cell lymphoma, 330
mycosisfungoides, 23
myelogenousleukemia, 397
Sézary syndrome, 124
T‐cell prolymphocyticleukemia, 419
T‐lymphoblastic lymphoma, 429

Stenotrophomonasmaltophilia infection, 543
steroidsintralesional

cutaneous and systemic plasmacytosis, 535
lymphocytoma cutis, 523
nodular scabies, 504

pre‐tibiallymphoplasmacytic plaque in
children, 487

pseudolymphoma in tattoo, 524
steroids local

intralymphatichistiocytosis, 541
lichenaureus, 488
lymphomatoidpapulosis, 147
mycosisfungoides, 20, 28, 38, 488
pagetoidreticulosis localized, 83

steroids systemic
actinicreticuloid, 497
angioimmunoblastic T‐cell lymphoma, 246
Castleman disease, 537
histiocytoid Sweet syndrome, 539
IgG4‐related disease, 537
marginal zone lymphoma, 286
pseudolymphomas in HIV patients, 500
pseudolymphomas in lupus

erythematosus, 515
psoriasis, 497
subcutaneouspanniculitis‐like T‐cell

lymphoma, 187
T‐cell large granular lymphocytic

leukemia, 422
subcutaneous anaplastic large cell lymphoma,

156, 185
subcutaneouspanniculitis‐like T‐cell

lymphoma
associated disorders

autoimmune disorders, 181, 187
lupuserythematosus, 468
lupuspanniculitis, 181, 185, 187, 191,

511–513
children/adolescents, 181
clinical features, 180
definition and diagnostic criteria problems,

187
differential diagnosis

cutaneous NK/T‐cell lymphomas with
subcutaneous tissue involvement, 13

γ/δ T‐cell lymphoma cutaneous, 26,
195, 201

lupuspanniculitis, 181, 185, 187, 511–513
panniculitis in children with immune

deficencies, 181
pseudolymphomatous infiltrates in

immunodeficiency, 511
etiology and pathogenesis, 181
hemophagocytic syndrome in, 181, 184, 187
histopathology, 204
immunohistology, 183
molecular genetics, 183–184
post‐transplant, 361
prognosis, 187
treatment, 187

surgicalartifatcs
cauterizationartifacts, 4
crush‐artifacts, 4, 444

dryingartifacts, 4, 6
fixationartifacts, 4, 262, 444
harmonic blades, 4

surgical techniques, 4
surgical treatment

anaplastic large cell lymphoma, 150–154
B‐cell chronic lymphocytic leukemia, 385
CD4+ small‐medium T‐cell

lymphoproliferative disorder, 276
CD30+ T‐cell pseudolymphomas, 502
folliclecenter lymphoma, 255, 268
localizedpagetoidreticulosis (Woringer‐

Kolopp), 81
lymphocytoma cutis, 518, 520
marginal zone lymphoma, 11, 275, 276,

276, 277
plasma cell granuloma, 535
scabies nodular, 502–503
solitary idiopathic B/T‐cell

pseudolymphoma, 481
tattoospseudolymphomatous, 524
T‐cell/histiocyte‐rich B‐cell lymphoma

cutaneous, 346
Sweet’s syndrome, 391, 400

“histiocytoid” (see histiocytoid Sweet’s
syndrome)

syphilispseudolymphomatous, 357, 529, 534
histopathology, 529, 530

syringolymphoid hyperplasia with alopecia
54, 61

syringometaplasia, 61, 73, 74, 99
syringotropic, mycosis fungoides, 29, 53, 54,

60–61, 71, 72, 73, 74, 99
systemicplasmacytosis see plasmacytosis

tacrolimus topical
actinicreticuloid, 478
lymphocytoma cutis, 518

talimogenelaherparepvec (T‐VEC), 277, 515
tattoospseudolymphomas in, 476, 524
tazarotene mycosis fungoides, 82
T/B‐cell angiomatoidpseudolymphoma,

486–487, 487, 488
T‐cell/histiocyte‐rich large B‐cell lymphoma

cutaneous
histopathology and immunophenotype,

345–346
molecular genetics, 345–346
treatment and prognosis, 346

T‐cell large granular lymphocytic
leukemia, 422

T‐cell prolymphocyticleukemia (T‐PLL),
419–421, 420

T‐cell receptor (TCR)
gene rearrangement, 10, 11, 46, 53, 117, 148,

229, 236, 413, 479, 495, 512, 540, 542
silent (null‐cell), 201
Vβ repertoire, 24, 117

Page 584

584 Index

T‐cell‐rich angiomatoidpolypoidpseu­
dolymphoma, 486–487, 488

T‐cell‐rich B‐cell lymphoma see T‐cell/
histiocyte‐rich large B‐cell lymphoma
cutaneous

telangiectasia
intralymphatic CD30+ anaplastic large

T‐cell lymphoma, 168
intravascular large B‐cell lymphoma,

315, 316
poikilodermatous mycosis fungoides,

69, 72, 85
teleangiectatic mycosis fungoides, 69
testicular B‐cell lymphoma, 299
T follicular (TFH) cells

inangioimmunoblastic T‐cell lymphoma
(see angioimmunoblastic T‐cell
lymphoma)

CD4+ small‐medium T‐cell
lymphoproliferative disorder
cutaneous, 11

mycosisfungoides, 41–42, 122
peripheral T‐cell lymphoma

NOS, 225
Sézary syndrome, 41–42, 122
in T‐follicular helper (TFH)‐cell

lymphoma cutaneous (see T‐follicular
helper (TFH)‐cell lymphoma
cutaneous)

T‐follicular helper (TFH)‐cell lymphoma
cutaneous, 225

thalidomide
anaplastic large cell lymphoma, 164
IgG4‐related disease, 529
pseudolymphomas at sites of

vaccination, 524
therapy‐resistant dermatitis, 38

T‐lymphoblastic lymphoma cutaneous
clinical features, 428
histopathology and immunophenotype,

428–429
molecular genetics, 428–429
treatment and prognosis, 429

TNM classification cutaneous lymphomas, 153
tofacitinib, 478
total body irradiation

mycosisfungoides, 83, 88
Sézary syndrome, 113

transdifferentiation
cutaneous follicle center lymphoma, 255
myelogenousleukemia, 7, 390

T‐regulatory (Treg) cells
adult T‐cell leukemia/lymphoma, 43
lymphomatoidpapulosis, 43
mycosisfungoides, 43
peripheral T‐cell lymphoma NOS, 43
Sézary syndrome, 122

Treponemapallidum, 534, 534
trichophytiapseudolymphomatous, 504
triple‐hit lymphoma, 299
tumor vaccines mycosis fungoides, 86
T‐VEC see talimogenelaherparepvec

ulcero‐necrotic PLEVA see
pityriasislichenoides

ulcusrodens, 28–29, 33
ultraviolet light A (UV‐A) therapy see PUVA
ultraviolet light B (UV‐B) therapy

actinicreticuloid, 477, 477–478, 478
B‐cell chronic lymphocytic leukemia, 385
mycosisfungoides

follicular mycosis fungoides, 55, 57,
135, 481

unclassifiable cutaneous lymphoma, 566

vaccination, pseudolymphoma at sites of,
522–524, 527

varicella zoster virus infections, 506, 507
viral warts, pseudolymphoma at sites of, 502
viruses see specific viruses
vitiligopseudolymphomatous, 489–490, 492

Waldenströmmacroglobulinemia,
346–347, 348

warts, pseudolymphoma at sites of, 502
“watchful waiting” treatment strategy

atypical lymphoid proliferations, 567
folliclecenter lymphoma, 268
lymphomatoidpapulosis, 148
marginal zone lymphoma, 286
mycosisfungoides, 97

Weber‐Christian panniculitis, 180
WHO see World Health Organization

classification, 1, 2, 65, 67, 124, 158, 219,
235, 243, 247, 253, 274–276, 299, 310,
315, 326, 344, 390, 396, 407, 424, 427
(see also specific entities)

WHO‐EORTC classification, 4, 53, 347 see
also specific entities

Woringer‐Kolopptype, of mycosis fungoides,
53, 61–64, 74, 75, 81, 463 see also
pagetoid reticulosis

World Health Organization (WHO), xiii, 1,
13, 23, 113, 133, 180, 195, 219, 228,
235, 239, 243, 253, 255, 274, 299, 315,
326, 357, 390, 407, 424, 437, 481

xanthoerythrodermaperstans, 26, 28

zosteriform mycosis fungoides, 53
zoster, pseudolymphoma in, 379, 380,

504–506, 507

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