Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop.
OBJECTIVE
Aggressive epidermotropic cutaneous CD8(+) lymphoma is currently afforded provisional status in the WHO classification of lymphomas. An EORTC Workshop was convened to describe in detail the features of this putative neoplasm and evaluate its nosological status with respect to other cutaneous CD8(+) lymphomas.


RESULTS
Sixty-one CD8(+) cases were analysed at the workshop; clinical details, often with photographs, histological sections, immunohistochemical results, treatment and patient outcome were discussed and recorded. Eighteen cases had distinct features and conformed to the diagnosis of aggressive epidermotropic cutaneous CD8(+) lymphoma. The patients typically present with widespread plaques and tumours, often ulcerated and haemorrhagic, and histologically have striking pagetoid epidermotrophism. A CD8(+) /CD45RA(+) /CD45RO(-) /CD2(-) /CD5(-) /CD56(-) phenotype, with one or more cytotoxic markers, was found in seven of 18 patients, with a very similar phenotype in the remainder. The tumours seldom involve lymph nodes, but mucosal and central nervous system involvement are not uncommon. The prognosis is poor, with a median survival of 12 months. Examples of CD8(+) mycosis fungoides, lymphomatoid papulosis and Woringer-Kolopp disease presented the typical features well documented in the CD4(+) forms of those diseases.


CONCLUSIONS
Aggressive epidermotropic cutaneous CD8(+) lymphoma is a distinct lymphoma that warrants inclusion as a distinct entity in future revisions of lymphoma classifications.
St John's Institute of Dermatology, London, UK.
Alistair
A
Robson
Department of Dermatology, Charité-University Medicine, Berlin, Germany.
Chalid
C
Assaf
Department of Pathology, Universite Paris, Paris, France.
Martine
M
Bagot
Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland.
Gunter
G
Burg
St John's Institute of Dermatology, London, UK.
Eduardo
E
Calonje
CHRU, Lille, France.
Christine
C
Castillo
Department of Dermatology Medical, University of Graz, Graz, Austria.
Lorenzo
L
Cerroni
Department of Dermatology, University of L'Aquila, Rome, Italy.
Nicola
N
Chimenti
Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France.
Pierre
P
Dechelotte
Department of Pathology, University of Clermont-Ferrand, Clermont-Ferrand, France.
Frederic
F
Franck
Department of Dermatology, Ghent University Hospital, Gent, Belgium.
Maria
M
Geerts
Department of Dermatology, Charité-University Medicine, Berlin, Germany.
Sylke
S
Gellrich
Department of Pathology, Western General Hospital, Edinburgh, UK.
John
J
Goodlad
Department of Dermatology and Venereology, University of Zurich, Zurich, Switzerland.
Werner
W
Kempf
Department of Dermatology, University of Vienna, Vienna, Austria.
Robert
R
Knobler
Department of Dermatology Medical, University of Graz, Graz, Austria.
Cesare
C
Massone
Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands.
Chris
C
Meijer
Hospital Universitario, Universidad Complutense, Madrid, Spain.
Pablo
P
Ortiz
Departmentof Pathology, Dijon's University Hospital, Dijon, France.
Tony
T
Petrella
Division of Dermatology, University of Florence Medical School, Florence, Italy.
Nicola
N
Pimpinelli
Department of Dermatology, Charité-University Medicine, Berlin, Germany.
Joclim
J
Roewert
St John's Institute of Dermatology, London, UK.
Robin
R
Russell-Jones
Division of Pathological Anatomy, University of Florence, Florence, Italy.
Marco
M
Santucci
Department of Dermatology, Charité-University Medicine, Berlin, Germany.
Mattias
M
Steinhoff
Department of Dermatology, Charité-University Medicine, Berlin, Germany.
Wolfram
W
Sterry
Department of Pathology Henri-Mondor Hospital, University Paris-Val-de-Marne, Paris, France.
Janine
J
Wechsler
St John's Institute of Dermatology, London, UK.
Sean
S
Whittaker
Department of Dermatology, Leiden University, Leiden, the Netherlands.
Rein
R
Willemze
Department of Dermatology, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico and Università degli Studi di Milano-Bicocca, Milan, Italy.
Emilio
E
Berti
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology*; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D016410:Lymphoma, T-Cell, Cutaneous / Q000145:classification* / Q000175:diagnosis* / Q000276:immunology*; D008297:Male; D008875:Middle Aged
CD8 antigen; diagnosis; lymphoma; mycosis fungoides; prognosis