DefinitionThis section has been translated automatically.
Cutaneous lymphomas (CL) belong to the group of extranodal non-Hodgkin lymphomas. They are defined as progressive, heterogeneous, malignant neoplasias of the lymphatic system of unknown aetiology that originate from the skin or primarily affect the skin.
By definition, primary cutaneous lymphomas develop in the skin and usually remain confined to the skin organ for a long time (at least 6 months).
Secondary cutaneous lymphomas originate from disseminated, primarily nodal or extranodal lymphomas.
Primary CL comprise a broad, clinically and histologically heterogeneous spectrum of lymphoproliferative neoplasms, wherein about 70%-80% of cutaneous lymphomas can be assigned to cutaneous T-cell lymphomas, about 15-20% to cutaneous B-cell lymphomas and +/-10% to other, rare forms of cutaneous lymphomas.
In principle, cutaneous lymphomas (CL) can be equated with "nodal" (starting from systemic lymphomas). However, the particularities of the skin-specific terrain (recirculation, homing phenomena, tropism of the T-lymphocytes for structures of the surface and adnexal epithelia) have to be taken into account which modify the specifications of the Kiel classification which has been in existence since the seventies of the last century.
The EORTC Classification and the current WHO Classification describe nosological entities defined by clinical, histo- and cytomorphological as well as phenotypic and genotypic features. Since the EORTC classification better reflects the diversity and specificity of cutaneous lymphomas than the current WHO classification, it is recommended to use both classifications in parallel (Level of evidence IV).
The "nodal" classifications of non-Hodgkin lymphomas are transferable to cutaneous lymphomas (CL) with regard to their basic division into B- and T-cell lymphomas. Thus, all current classifications of CL follow this dualistic principle with differentiation into:
- Cutaneous T-cell lymphomas (CTCL) - about 70% of all cutaneous lymphomas
- Cutaneous B-cell lymphomas (CBCL) - about 20% of all cutaneous lymphomas
Neoplasias of unclear line assignment and differentiation (CD4+ CD65+haematodermal neoplasia - blastic NK-cell lymphoma)
Other rare cutaneous lymphomas - about 10% of all cutaneous lymphomas
For all cutaneous lymphomas a classification principle according to:
- Primary cutaneous lymphomas (no other organ manifestation at the time of diagnosis)
- Secondary cutaneous lymphomas.
This subdivision concerns primarily the cutaneous B-cell lymphomas and only secondarily the cutaneous T-cell lymphomas. This differentiation principle has fundamental diagnostic and therapeutic significance.
With regard to their biological characteristics, cutaneous lymphomas are also divided according to prognostic and cytological/immunohistological criteria into:
- Lymphomas with good prognosis (indolent cutaneous lymphomas; mean survival time > 5 years)
- Lymphomas with moderate prognosis (mean survival time 2-5 years)
- Lymphomas with poor prognosis (aggressive cutaneous lymphomas; mean survival time < 2 years).
Less uniform, however, are the subdivisions of CTCL. Thus, the present classifications mainly refer to the low-grade T-cell lymphoma of the mycosis fungoides type and some subspecificities as well as its (generally clinically severe) erythrodermal-leukemic variant, the Sézary syndrome. Both clinical pictures are also recognized as entities in the different nodal classifications. This results in a frequency principle for cuntaneous T-cell lymphomas (CTCL) according to general clinical aspects, which leads to the following (mental) subdivision:
- Cutaneous T-cell lymphomas of the mycosis fungoides type
- Cutaneous T-cell lymphomas of the "non-mycosis fungoides" type.
Some lymphomas cannot be classified immunologically due to missing surface markers. In many cases, immunohistochemical and molecular biological methods (see CD-classification) allow the correct phenotyping, classification and in some cases prognosis.
ClassificationThis section has been translated automatically.
WHO-EORTC Classification of cutaneous lymphomas Cutaneous T-cell and NK-cell lymphomas
Cutaneous T-cell lymphomas
- Mycosis fungoides (MF)
- Mycosis fungoides variants and subtypes
- Sézary syndrome (SS)
- Adult T-cell leukemia/lymphoma (HTLV+)
- Primary cutaneous CD30+ lymphoproliferative diseases
- Subcutaneous panniculitis-like T-cell lymphoma (SPTL)
Extranodal NK/T cell lymphoma, nasal type
- Primary cutaneous peripheral T-cell lymphoma, not further specified (NOS)
- Primary Cutaneous δ/δ- T-Cell Lymphoma
- Aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma
- Primary cutaneous CD4+ small to medium T-cell lymphoproliferation
- Primary cutaneous acral CD8+ T-cell lymphoma
Cutaneous B-cell lymphomas
- Primary cutaneous follicular center lymphoma (PCFCL)
- Primary cutaneous marginal zone lymphoma (PCMZL)
- Primary cutaneous diffuse large-cell B-cell lymphoma-leg type (PCBLT)
- EBV-positive mucocutaneous ulcer
- EBV-positive diffuse large cell B-cell lymphoma (not further specified)
- Primary cutaneous intravascular large B-cell lymphoma
- Haematological precursor neoplasias
- Blastocytic neoplasia of plasmacytoid dendritic cells
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Occurrence/EpidemiologyThis section has been translated automatically.
Next to the gastrointestinal tract, the skin is the most common manifestation organ of malignant non-Hondgkin lymphomas, with an incidence of 0.5-1.0/100,000 inhabitants/year.
In larger studies, mycosis fungoides is found most frequently (about 50% of all cutaneous lymphoma cases), followed by primary cutaneous follicular center lymphoma (16.9%) and lymphomatoid papulosis (15.9%).
ManifestationThis section has been translated automatically.
CL low malignancy almost never occurs in patients <20 years of age. CL of high malignancy show 2 manifestation peaks: 5-15th LJ and 60-70th LJ. The average age at onset of the disease is >50 years, with the exception of patients with lymphomatoid papulosis. Patients with primary cutaneous diffuse large cell B-cell lymphoma have an average age of 80 years (Nashan D et al. 2018).
In cutaneous T-cell lymphomas, male patients predominate with about 75%. The mean age of onset of mycosis fungoides is 60 years (20-90 years).
Indolent BCL - m:w=1:1
Clinical featuresThis section has been translated automatically.
Basically, unspecific and specific skin changes can be observed in lymphomas. Non-specific skin changes are hidden under eczematous, lichenoid or ichthyosiform, often pruriginous, exanthematic clinical pictures.
The cutaneous B-cell lymphomas appear rather monomorphic in their clinical and histological manifestation, characterized by mostly indolent, solitary but also disseminated, smooth, red to brown-red papules and nodules with nodular dermal and subcutaneous infiltrates with intact epidermis and mostly free border strip.
In contrast, the clinical and histological picture of cutaneous T-cell lymphomas is extremely varied and ranges from "ichthyosis-like" mucinosis follicularis, parapsoriasis en plaques with their extensive psoriasiform or eczematous plaques, to erythroderma. Ulcerated or non-ulcerated, small papular or large nodular lesions (0.5 cm to 15.0 cm) are also found. The lesions are solitary or disseminated, they tend to confluence. Not infrequently an arrangement aligned according to the cleavage lines of the skin is observed. This makes a clear clinical and histological classification extremely difficult and requires a lot of personal experience.
Differential diagnosisThis section has been translated automatically.
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LiteratureThis section has been translated automatically.
- Apisarnthanarax N et al (2002) Treatment of cutaneous T cell lymphoma: current status and future directions. Am J Clin Dermatol 3: 193-215
- AWMF Guidelines Report (2014) WHO-EORTC Classifications of Cutaneous Lmyphoma. S.4
- Dippel E et al (2018) S2k guidelines -cutaneous lymphomas update 2016-part2: treatment and follow up. J Dermatol Ges 16: 112-122
- Gellrich S et al (2000) Cutaneous B-cell lymphoma. dermatologist 51: 363-373
- Hallermann C et al (2011) Survival data for 299 patients with primary cutaneous lymphomas: a monocentre study. Acta Derm Venereol. 2011 91:521-525
- Kotz EA et al (2003) Cutaneous T-cell lymphoma. J Eur Acad Dermatol Venereol 17: 131-137
- Kerl H, Volkenandt M, Cerroni L (1994) Malignant lymphomas of the skin. dermatologist 45: 421-443
- Nashan D et al (2018) Primary cutaneous lymphoma - a case series of 163 patients. Dermatologist 69: 1014-1020
- Willemze R, Meijer CJ (2000) EORTC classification for primary cutaneous lymphomas: a comparison with the R.E.A.L. Classification and the proposed WHO Classification. Ann Oncol 11: 11-15
- Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785
Incoming links (31)Basidiomycosis; Biopsy; Borrelia lymphocytoma; Chop scheme; Cl; Copp scheme; Cop scheme; Cp scheme; Cutaneous lymphoma; Disseminated sporotrichosis; ... Show all
Outgoing links (22)Adult t-cell lymphoma/leukemia (htlv+); Cd classification; Cutaneous b-cell lymphomas (overview); Cutaneous t-cell lymphomas (overview); Cytostatic drugs, supportive therapy; Erythroderma; exfoliative dermatitis;; Follicular mucinosis; Folliculotropic mycosis fungoides; Granulomatous slack skin; Lymphoma cutaneous nk/t cell lymphoma (overview); ... Show all
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