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Cutaneous Lymphomas: An Update. Part 2 B-Cell Lymphomas and Related Conditions

Kempf, Werner MD*,†; Kazakov, Dmitry V. MD, PhD; Mitteldorf, Christina MD§

The American Journal of Dermatopathology: March 2014 - Volume 36 - Issue 3 - p 197–210
doi: 10.1097/DAD.0b013e318289b20e
CME Article

Abstract: Primary cutaneous B-cell lymphomas (PCBCL) are the second most common form of primary cutaneous lymphomas and account for approximately 25%–30% of all primary cutaneous lymphomas. Both forms of low-grade malignant PCBCL, primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma of mucosa-associated lymphoid tissue-type (MALT lymphoma) (PCMZL) represent the vast majority of PCBCL and show an indolent slowly progressive course and an excellent prognosis despite a high recurrence rate. Genetic analysis indicates that PCMZL differ from other forms of extranodal MALT lymphomas. The more common class-switched and the non–class-switched form of PCMZL can be distinguished as two distinctive subsets that differ in the cellular composition, IgM expression, and biological behavior with extracutaneous involvement found in the non–class-switched form. Recently, unusual clinical and histological forms of PCMZL and PCFCL manifesting with miliary or agminated lesions have been described that are diagnostically challenging. In contrast to PCMZL and PCFCL, primary cutaneous diffuse large B-cell lymphoma, leg type, and other rare forms of large B-cell lymphomas such as intravascular large B-cell lymphoma have an unfavorable prognosis. There is an emerging group of Epstein–Barr virus (EBV)–driven B-cell lymphoproliferations including posttransplant lymphoproliferative disorders and mucocutaneous ulcer occurring in immunocompromised patients and EBV-associated diffuse large B-cell lymphoma of the elderly arising in the setting of senescence-linked immunodeficiency. This review reports on recent findings expanding the spectrum of clinicopathological features, differential diagnostic aspects, and the pathogenesis of PCBCL and discusses the group of EBV-associated B-cell lymphoproliferations involving the skin.

*Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland;

Professor, Department of Dermatology, University Hospital Zürich, Zürich, Switzerland;

Professor, Department of Pathology, Medical Faculty in Pilsen, Pilsen, Charles University in Prague, Czech Republic; and

§Doctor, Department of Dermatology, Klinikum Hildesheim GmbH, Hildesheim, Germany.

Reprints: Werner Kempf, MD, Kempf und Pfaltz, Histologische Diagnostik, Seminarstrasse 1, CH-8042 Zürich, Switzerland (e-mail:

All authors and staff in a position to control the content of this CME activity and their spouses/life partners (if any) have disclosed that they have no financial relationships with, or financial interests in, any commercial organizations pertaining to this educational activity.

© 2014 by Lippincott Williams & Wilkins.