Claus-Detlev Klemke received travel support for scientific conferences and lecture fees for scientific presentations from for TEVA/Cephalon Pharma GmbH and Therakos, Johnson & Johnson Medical GmbH. He is a member of the TEVA Cutaneous Lymphoma Advisory Board.
Article first published online: 7 JAN 2014
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.
JDDG: Journal der Deutschen Dermatologischen Gesellschaft
Volume 12, Issue 1, pages 7–30, January 2014
How to Cite
Klemke, C.-D. (2014), Cutaneous lymphomas. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 12: 7–30. doi: 10.1111/ddg.12237
Conflicts of interest
Section Editor: Prof. Dr. Jan C. Simon, Leipzig
Primary cutaneous lymphomas are the second most common type of extranodal non-Hodgkin lymphomas (NHL).
The two main groups are primary cutaneous T-cell lymphoma (CTCL) and primary cutaneous B-cell lymphoma (CBCL).
When diagnosing cutaneous lymphoma, one must insure that the lymphoma is limited to the skin at the time of diagnosis.
The prognosis and treatment of cutaneous lymphomas differs from nodal NHL.
The WHO-EORTC classification of ≠cutaneous lymphomas allows cutaneous lymphomas to be roughly divided into those with a good, moderate, or poor prognosis.
Cutaneous lymphoma may be diagnosed using a diagnostic ladder which includes the following features: clinical appearance, histology, immunohistology, and molecular biology.
In patients with clinically palpable lymph nodes located near the skin's surface, an enlarged node should be removed to determine potential lymph node involvement.
To diagnose CTCL, molecular biological tests include a polymerase chain reaction (PCR) test to determine if there is gene rearrangement in the T-cell receptor chain (TCR-gamma PCR).
Molecular biological diagnosis based on the BIOMED-2′ protocol allows for molecular staging of the lymph nodes, the skin, blood, and bone marrow 
Staging takes into account various stages of cutaneous and lymph node involvement as well as an assessment of the blood and visceral organs.
Most patients have patches or plaques, which may persist for years or even decades.
Thus, in inflammatory skin disorders of uncertain origin, MF should always be included in the differential diagnosis
In many patients with MF, definitive diagnosis is often only possible after a few years
The chief clinical symptom which usually causes the patient to consult a dermatologist is erythroderma.
In terms of clinical course, Sézary syndrome is much more aggressive than MF, and it is usually associated with very intense and often torturous pruritus.
Patients have solitary or, occasionally, multiple papules which have a tendency toward central ulceration. A typical characteristic is the healing of individual lesions.
Most patients have solitary or localized nodules or tumors
Patients with hemophagocytic syndrome have a significantly poorer prognosis than those who do not.
The disease must be differentiated from the above-mentioned subcutaneous panniculitis-like T-cell lymphoma with an α-β tumor cell phenotype.
Patients usually have multiple plaques or tumors on the trunk or extremities as well as involvement of the nasal cavities or oral mucosa.
Histological confirmation of the diagnosis and the differentiation between PCFCL and cutaneous involvement in nodal NHL are essential.
Unlike PCFCL, patients with this type of lymphoma have a tendency develop extracutaneous involvement; the prognosis is considerably worse.
The treatment of cutaneous lymphoma depends upon the specific entity and stage of disease .
Precise diagnosis is essential for adequate treatment.
In patients with Sézary syndrome, the treatment of choice is ECP, which should generally be combined with interferon-α and/or bexarotene as well as PUVA therapy.
Since some patients with lymphomatoid papulosis may experience spontaneous remission, one option is clinical observation without further treatment.
The treatment of rare cutaneous lymphomas is always based on the patient's specific characteristics. It is impossible to make standardized recommendations.
Accompanying psycho-oncologic care of patients is also very important. Patients often have great difficulty understanding these rare diseases and dealing with them.
- Issue published online: 7 JAN 2014
- Article first published online: 7 JAN 2014
- Manuscript Accepted: 15 SEP 2013
- Manuscript Received: 18 JUN 2013
Primary cutaneous lymphomas are extranodal non-Hodgkin lymphomas. They are classified into the two main groups of primary cutaneous T- and B-cell lymphomas. Very rare cases are derived from NK or plasmacytoid dendritic cells. The annual incidence is 1/100,000. Two-thirds of the patients have primary cutaneous T-cell lymphoma, and the remaining one-third have primary cutaneous B-cell lymphoma. Over the years, tremendous progress has been made regarding the diagnostics and classification of cutaneous lymphomas. An exact classification of cutaneous lymphomas is of great importance for the patient, because the different skin lymphomas have very different prognoses and require different therapeutic regimens.
The basis for making a diagnosis is a clinical-pathological correlation, including the use of several immunohistochemical markers and molecular biological methods. Treatment of cutaneous lymphoma is adapted to the type of lymphoma and disease stage. First-line therapy consists of treatments that target the skin. Systemic treatment is used in advanced disease. Many targeted therapies have been introduced into routine clinical care in recent years. This review presents an up-to-date approach to the diagnosis and treatment of primary cutaneous lymphomas.