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G. BURG P. E. LEBOIT (EDS.)
Unusual Cases
G. BURG P. E. LEBoIT (EDS.)
Cutaneous Lymphomas Unusual Cases Co-Editors
W. KEMPF and B. MULLER
WITH 328 COLOUR FIGURES
GUNTER BURG, M.D.
Professor of Dermatology and Venerology Department of Dermatology University Hospital of Ziirich Gloriastrasse 31 8091 Ziirich/Switzerland
PHILIPP LEBOIT, M.D.
Professor of Dermatopathology, University of California 1701 Divisadero St., Room 350, CA 94115, San Francisco, USA
WERNER KEMPF, M.D.
BEATRIX MULLER, B.S.
Department of Dermatology University Hospital of Ziirich Gloriastrasse 31 8091 Ziirich/Switzerland
ISBN 978-3-642-63314-0 ISBN 978-3-642-57624-9 (eBook) DOI 10.1007/978-3-642-57624-9
Die Deutsche Bibliothek - CIP-Einheitsaufnahme A catalogue record for this publicat ion is available from Die Deutsche Bibliothek
This work is subject to copyright. Ali rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permis sion for use must always be obtained from Steinkopff-Verlag. Violations are liable for prosecution under the German Copyright Law.
© Springer-Verlag Berlin Heidelberg 2001
Originally published by Steinkopff-Verlag Darmstadt in 2001
The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.
Product liability: The publishers cannot guarantee the accuracy of any information about the application of operative techniques and medications contained in this book. In every individual case the user must check such information by consulting the relevant literature.
Production: Klemens Schwind Cover-Design: Erich Kirchner, Heidelberg Typesetting: K + V Fotosatz GmbH, Beerfelden
SPIN 10760042 105/7231-5 4 3 2 1 O - Printed on acid-free paper
I Preface
Cutaneous lymphomas are one of the most difficult but also most interesting and challenging areas in clinical and experimental dermatology and pathology. While even "routine" cases can pose diagnostic difficulties beyond those associated with similar lymphomas in lymph nodes, cutaneous lymphomas often have unusual features that pose a wide spectrum of questions concerning diagnosis, classification, pathogenesis and therapy.
This book is an unique collection of unusual cases of cutaneous T-cell, B-cell and NK-cell lymphomas, leukemias and other disorders related to cutaneous lymphoma, presented in a concise format. The authors have provided excellent illustrations and precise descriptions with short comments and reliable references. All of the authors are highly regarded experts in the field of cutaneous lymphomas and lymphoproliferative diseases and their contributions guarantee the high quality performance of this book. The limitations of their perceptions of these cases reflect the limits of our knowledge in 2001, not the limitations of their expertise.
Zurich and San Francisco, February 2001
GUNTER BURG
PHILIP LEBoIT
I Table of Contents
1 Cutaneous T-cell Lymphoma (an)
• 1.1 Mycosis Fungoides and Related Disorders . . . . . . . . . . . . . . . . . . . 2
Solitary mycosis fungoides ......... . .. . ... . ........ . ......... 2 1. CERRONI and H. KERL
Unilesional mycosis fungoides E. HODAK
4
Small cell variant of mycosis fungoides ........................ 6 P. V. M. M. DIEDEREN, C. W. VAN HASELEN, J. TOONSTRA,
S.c.J. VAN DER PUTTE, 1.H.P.M. RADEMAKERS, J.A.J. FABER,
and W.A. VAN VLOTEN
Mycosis fungoides and idiopathic CD4+ T-Iymphocytopenia . . . . . . . 8 B. DRENO
Folliculotropic T-cell lymphoma with self-resolving eosinophil-rich lymphomatoid nodule .... . . . ... . ....... ..... .... . .... .. .. . . . 10 s. KOSSARD
Follicular cutaneous T-cell lymphoma (follicular mycosis fungoides) 12 E. HODAK
Mycosis fungoides/Sezary/s syndrome presenting as generalized follicular mucinosis ........ . .. . ....... 14 P.E. LEBOIT
Dyshidrotic mycosis fungoides ................ . ......... .. .... 16 1. CERRONI and H . KERL
Ichthyosiform mycosis fungoides ... ......... . ... . ...... .. . .. .. 18 E. HODAK
Lichenoid purpuric mycosis fungoides P.E. LEBOIT
20
VIII • Table of Contents _____________ 1XlIX; __ ~"'""'"I'_~_~~~
• Granulomatous mycosis fungoides ............................ 22 B. DRENO and B. BUREAU
• Granulomatous slack skin in a patient with mycosis fungoides . . . . 24 C. W. VAN HASELEN, J. TOONSTRA, and W. A. VAN VLOTEN
• Granulomatous slack skin P.E. LEBOIT
• 1.2 Sezary's Syndrome and Related Disorders
• Cutaneous T-cell lymphoma presenting as an erythroderma
26
28
with generalized hyperpigmentation and highly elevated serum IgE 28 S. A. BUCHNER
• Sezaris syndrome and human immundeficiency virus negative Kaposi's sarcoma 30 B. DRENO and A. KHAMMARI
• Clone-negative CD8+ Sezary syndrome -
•
a prominent leukemic variant of erythrodemic actinic reticuloid .. 32 K.IwATSUKI
Clonal granulomatous infiltrates in Sezary's syndrome W. KEMPF, R. DUMMER, and G. BURG
34
• 1.3 CD30-positive Lymphoproliferative Disorders . . . . . . . . . . . . . . . . 36
• Regional lymphomatoid papulosis ............................ 36 J. J. SCARISBRICK and R. RUSSELL-JONES
• Primary cutaneous anaplastic large-cell CD30+ lymphoma in a child 38 S. A. BUCHNER
• Primary cutaneous CD30+ large-cell lymphoma with natural killer-cell phenotype and the t(2;5) translocation 40 G. S. WOOD, J. W. GOULD, and A. C. GILLIAM
• 1.4 Cytotoxic NK/T-cell Lymphomas and Related Disorders. . . . . . . . 42
• Primary cutaneous CD8+ T-cell lymphoma (mycosis fungoides-like type) ................................ 42 W. KEMPF, G. BURG, and R. DUMMER
• CD8+ cutaneous T-cell Iymphoproliferative disorder ............. 44 N. APISARNTHANARAX, D. JONES, and M. DUVIC
• Aggressive CD8+ epidermotropic cytotoxic T-cell lymphoma. . . . . . 46 D. MASSI, N. PIMPINELLI, and M. SANTUCCI
Table of Contents • IX
NK/T-cell C04+ CD56+ cutaneous lymphoma D. MASSI, N. PIMPINELLI, and M. SANTUCCI
Primary cutaneous Natural-killer cell lymphoma A. DARVAY, K.J. WAITE, and R. RUSSEL-JONES
Hydroa vacciniforme; a T-cell proliferative disorder
48
50
associated with latent EB virus infection ....................... 52 K.lwATSUKI
• 1.S Other Cutaneous T-cell Lymphomas 54
Subcutaneous panniculitis-like T-cell lymphoma M. FLAIG and C. A. SANDER
Subcutaneous panniculitis-like T-cell lymphoma c.J.G. SANDERS and W.A. VAN VLOTEN
T-zone lymphoma with involvement of the skin w. KEMPF and G. BURG
Cutaneous peripheral T-cell lymphoma
54
56
58
presenting as sudden fever and rash .......................... 60 N. APISARNTHANARAX, J. L. MADEIROS, and M. DUVIC
T-cell signet-ring cell proliferation of the skin. . . . . . . . . . . . . . . . . . . 62 J. TOONSTRA and S. C. J. VAN DER PUTTE
B-cell-rich cutaneous T-cell lymphoma ......................... 64 M. MORI, N. PIMPINELLI, and M. SANTUCCI
2 Cutaneous B-cell lymphoma (CBCL)
• Cutaneous B-Iymphoblastic lymphoma . ....................... . 68 L. CERRONI and H. KERL
Precursor B-Iymphoblastic lymphoma with skin involvement 70 W. KEMPF and G. BURG
Disseminated lymphocytoma cutis with conjunctival lesions . . . . . . . 72 J.J. SCARISBRICK and R. RUSSEL-JONES
Cutaneous B-cell lymphoma (MALT) associated with Borrelia burgdorferi infection c. SCHULZ, F. HAGEMEISTER, and M. DUVIC
74
• Large B-cell lymphoma of the leg recurring as intravascular large-cell lymphoma ........................................ 76 G.S. WOOD, N. KAMATH, and A.C. GILLIAM
x • Table of Contents
Cutaneous B-cell lymphoma and benign cutaneous hyperplasia associated with anetoderma . . . . . . . . . . . . . . . . . . . . . . . 78 A. BouLoc, A. COSNES, J. WECHSLER, and M. BAGOT
Reversible lymphoma due to methotrexate therapy in a patient with rheumatoid arthritis .. .. .. .... .. .. .. ........ 80 P.E. LEBOIT
3 Leukemia
•
•
B-cell chronic lymphocytic leukemia at the site of herpes simplex scar ............................ 84 L. CERRONI and H. KERL
Leukemia cutis in acute monocytic leukemia S. A. BUCHNER
CD56+ acute myeloid leukemia
86
with involvement of the skin ...... . ... . ... . .. . ......... . ... . 88 H. KUWABARA, M. NAGAI, and T. NUMAHARA
Adult T-cell lymphoma/leukemia M. J. FLAIG, and C. A. SANDER
A cutaneous variant of adult T-cell leukemia
90
mimicking pityriasis lichenoides et varioliformis acuta (PLEVA) 92 K. IWATSUKI
Aleukemic leukemia cutis C. KENDRICK, N. APISANTHANARAX, O. ESTAILLA, D. JONES,
and M. DUVIC
Other Disorders Related to Cutaneous Lymphoma
94
Sinus histiocytosis with lymphadenopathy (Rosai-Dorfman disease) 98 D. INNOCENZI
Primary cutaneous Rosai-Dorfman disease with fatal outcome 100 J. RAMSAUER and W. MEIGEL
Kikuchi's disease involving lymph node and skin P.E. LEBOIT
Angiolymphoid hyperplasia with eosinophilia
102
and clonal T-cell population ....... .. .. _........ . ............. 104 W. KEMPF, A. C. HAEFFNER, R . G. PANIZZON, and G. BURG
Lymphoepithelioma-like carcinoma of the skin A. C. LIND and M. R. WICK
106
List of Authors
APISARNTHANARAX NARIN Dermatology Clinical Research Fellow The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
BAGOT MARTINE, M.D. Henri Mondor Hospital Department of Dermatology 51, Avenue du Marechal de Lattre de Tassigny 94010 Creteil/France
BouLOC ANNE, M.D. Henri Mondor Hospital Department of Dermatology 51, Avenue du Marechal de Lattre de Tassigny 94010 Creteil/France
BUCHNER STANISLAW, M.D. Professor Department of Dermatology University of Basel Peters graben 4 4031 Basel/Switzerland
BUREAU B., M.D. Department of Dermatology Hospital Hotel-Dieu PI. A. Ricordeau 44093 Nantes Cedex lIFrance
BURG GUNTER, M.D. Professor of Dermatology and Venerology Department of Dermatology University Hospital of Zurich Gloriastrasse 31 8091 Zurich/Switzerland
CERRONI LORENZO, M.D. Department of Dermatology University of Graz Auenbruggerplatz 8 8036 Graz/ Austria
COSNES A., MD Henri Mondor Hospital Department of Dermatology 51, Avenue du Marechal de Lattre de Tassigny 94010 Creteil/France
DARVAY A., MRCP Skin Tumour Unit St. John's Institute of Dermatology St. Thomas' Hospital Lambeth Palace Road London SEI 7EH
DAVID, MICHAEL, M.D. Rabin Medical Center Department of Dermatology Beilinson Campus Tel-Aviv University 49100 Petah Tiqva, Israel
DIEDEREN P.V.M.M., M.D. Dermatology and Venerology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
DRENO BRIGITTE, M.D. Department of Dermatology Hospital Hotel-Dieu PI. A. Ricordeau 44093 Nantes Cedex lIFrance
DUMMER REINHARD, M.D. Associate Professor Department of Dermatology University Hospital of Zurich Gloriastrasse 31 8091 Zurich/Switzerland
DUVIC MADELEINE, M.D. The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
ESTAILLA 0., M.D. The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
XII • List of Authors
FABER J.A.J., M.D. Dermatology and Venerology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
FEINMESSER, MEORA, M.D. Rabin Medical Center Department of Pathology Beilinson Campus Tel-Aviv University 49100 Petah Tiqva, Israel
FLAIG M.J., M.D. Department of Dermatology Ludwig-Maximilians-University Frauenlobstr. 9-11 80337 Munchen/Germany
GILLIAM A.C., M.D. Department of Dermatology University Hospitals/Ireland Cancer Center Case Western Reserve University Bolwell Health Center 11100 Euclid Avenue/Suite 3100 Cleveland, Ohio 44106-5028/USA
GOULD J.W., M.D. Department of Dermatology University Hospitals/Ireland Cancer Center Case Western Reserve University Bolwell Health Center 11100 Euclid Avenue/Suite 3100 Cleveland, Ohio 44106-5028/USA
HAFFNER ANDREAS C., M.D. Department of Dermatology University Hospital of Zurich Gloriastrasse 31 8091 Zurich/Switzerland
HAGEMEISTER F., M.D. The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
HODAK EMMILIA, M.D. Rabin Medical Center Department of Dermatology Beilinson Campus Tel-Aviv University 49100 Petah Tiqva, Israel
INNOCENZI DANIELE, M.D. University of Rome "La Sapienza" Department of Dermatology V.le del Policlinico, 155 00161 Rome/Italy
IWATSUKI KEIJI, M.D. Department of Dermatology Fukushima Medical University School of Medicine I-Hikarigaoka, Fukushima 960-1295 Japan
JONES D., M.D. The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
KAMATH N., M.D. Department of Dermatology University Hospitals/Ireland Cancer Center Case Western Reserve University Bolwell Health Center 11100 Euclid Avenue/Suite 3100 Cleveland, Ohio 44106-5028/USA
KEMPF WERNER, M.D. Department of Dermatology Dermatopathology Unit University Hospital of Zurich Gloriastrasse 31 8091 Zurich/Switzerland
KENDRICK C., M.D. The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
KERL HELMUT, M.D. Department of Dermatology University of Graz Auenbruggerplatz 8 8036 Grazl Austria
KHAMMARI AMIR, PH.D. Department of Dermatology Hospital H6tel-Dieu PI. A. Ricordeau 44093 Nantes Cedex lfFrance
KOSSARD STEVEN, M.D. FACD Ph.D. Associate Professor of DermatologySkin & Cancer Foundation Australia 277 Bourke Street, Darlinghurst N.S.W. Australia 2010
KUWABARA HIROKO Second Department of Pathology Kagawa Medical University 1750-1, Miki-cho, Kita-gun Kagawa, 761-0793, Japan
LEBOIT PHILIP, M.D. Professor of Clinical Pathology and Dermatology Dermatopathology Section University of California, San Francisco 1701 Divisadero St., STE 335 San Francisco, CA 94115 USA
LIND ANNE C., M.D. Assistant Professor Washington University School of Medicine at Washington University Medical Center Division of Anatomic Pathology Campus Box 8118 660 South Euclid Avenue St. Louis, Missouri 63110-1093 USA
MADEIROS J.L., M.D. The University of Texas M.D. Anderson Cancer Center Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
MASSI DANIELA, M.D. Dept. of Human Pathology and Oncology University of Florence Nedical School Viale G.B. Morgagni, 85 50134 Florence/Italy
MEIGEL WILHELM N., M.D. Department of Dermatology AUg. Krankenhaus St. Georg Lohmiihlenstr. 5 20099 Hamburg/Germany
MORI MOIRA, M.D. Dept. Dermatological Sciences University of Florence Via degli Alfani, 37 50121 Florence/Italy
MULLER BEATRIX, B.S. Department of Dermatology Laboratory of Dermatopathology University Hospital of Ziirich Gloriastrasse 31 8091 Ziirich/Switzerland
NAGAI MASAMI, M.D. First Department of Internal Medicine Kagawa Medical University 1750-1, Miki-cho, Kita-gun Kagawa, 761-0793/Japan
NUMAHARA TOSHIHIKO, M.D. Department of Dermatology Kagawa Medical University 1750-1, Miki-cho, Kita-gun Kagawa, 761-0793/Japan
List of Authors
PANIZZON RENATO G., M.D. Service de dermato-venereologie CHUV, Hopital Beaumont Av. De Beaumont 29 1011 Lausanne/Switzerland
PIMPINELLI NICOLA, M.D. Dept. Dermatological Sciences University of Florence Via degli Alfani, 37 50121 Florence/Italy
RADEMAKERS L.H.P.M., M.D. Dermatology and Venerology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
RAMSAUER JULIA, M.D.
•
Department of Dermatology Allgemeines Krankenhaus St. Georg Lohmiihlenstr. 5 20099 Hamburg/Germany
RUSSELL-JONES ROBIN, MA, FRCP Director, Skin Tumour Unit St. John's Institute of Dermatology St. Thomas' Hospital Lambeth Palace Road London SEI 7EH
SANDER CHRISTIAN, M.D. Department of Dermatology Ludwig-Maximilians-University Frauenlobstr. 9-11 80337 Miinchen/Germany
SANDERS CORNELIUS J.G., M.D. Department of Dermatology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
SANTUCCI MARCO, M.D. Dept. of Human Pathology and Oncology University of Florence Medical School Viale G.B. Morgagni, 85 50134 Florence/Italy
SCARLSBRICK JULIA, MRCP Dermatology The Middlesex Hospital Mortimer Street London WIN 8AA
SCHULZ C., M.D. The University of Texas M.D. Anderson Cancer Center
XIII
Department of Internal Medicine Specialities Section of Dermatology 1515 Holcombe Boulevard-Box 28 Houston, TX 77030-4095 USA
XIV • List of Authors
TOONSTRA J., M.D. Dermatology and Venerology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
VAN DER PUTTE S.C.J., M.D. Dermatology and Venerology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
VAN HASELEN C.W., M.D. Dermatology and Venerology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
VAN VLOTEN WILLEM A., M.D. Department of Dermatology University Medical Center Heidelberglaan 100 3508 GA Utrecht/The Netherlands
WAITE K.J., M.D. Dermatology/Histopathology Skin Tumour Unit St. John's Institute of Dermatology St. Thomas' Hospital Lambeth Palace Road London SEI 7EH
WECHSLER JANINE, M.D. Department of Dermatology Henri Mondor Hospital 51, Ave de Tassigny 94010 Creteil/France
WICK MARK R., M.D. Professor of Pathology University of Virginia Medical Center Division of Surgical Pathology, Box 800214 Charlottesville, Virginia 22908/USA
WOOD GARY S., M.D. University Stations Clinics Department of Dermatology 2880 University Avenue Madison, WI 53705/USA
