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Primary cutaneous B-cell lymphomas and blastic plasmacytoid dendritic cell neoplasm

a short, updated review (Part II)

Gulia, Andreaa,b; Massone, Cesareb

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Journal of the Egyptian Women's Dermatologic Society: May 2012 - Volume 9 - Issue 2 - p 65-71
doi: 10.1097/01.EWX.0000413195.67737.1b
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Primary cutaneous B-cell lymphomas are neoplasms of the immune system characterized by the cutaneous proliferation of B lymphocytes that primarily arise in the skin, without extracutaneous manifestations of the disease at presentation. Extracutaneous spread can be observed during the course of the disease 1–3. Secondary cutaneous localization of extracutaneous lymphomas and leukemias represent a metastatic localization of the disease. Complete staging investigations are essential to establish this distinction 4.

In 1997, the European Organisation of Research and Treatment of Cancer (EORTC) proposed the first classification of primary cutaneous lymphomas (PCL) 5–7. A joint World Health Organisation-EORTC classification for PCL was published in 2005 (Table 1) and it recognizes three specific entities of cutaneous B-cell lymphoma: cutaneous follicle center lymphoma (FCL), the cutaneous marginal zone lymphoma (CMZL), and the cutaneous diffuse large B-cell lymphoma, leg type (DLBCL-leg) 1. In the WHO classification of 2008, the marginal zone lymphoma (MZL) has been included within the extranodal MZL of mucosa-associated lymphoid tissue (MALT) 4. Moreover, a new third category of precursor of hematologic neoplasm including the blastic plasmacytoid dendritic cell neoplasm (BPDCN) has been introduced (Table 2).

Table 1
Table 1:
WHO-EORTC classification of primary cutaneous lymphomas 1
Table 2
Table 2:
Comparison of the WHO-EORTC classification of 2005 and WHO classification of 2008 of primary cutaneous lymphomas 2

Primary cutaneous B-cell lymphomas

Primary cutaneous follicle center lymphoma

Primary cutaneous follicle center lymphoma (PCFCL) is defined as the neoplastic proliferation of germinal center cells confined to the skin by the absence of extracutaneous manifestations after complete staging investigations 1,8,9.

Clinical features

Patients are adults with erythematous papules, plaques, and tumors, usually nonulcerated, located mostly on the head, neck, and the trunk (Fig. 1). A miliary variant simulating rosacea or acne on the face has been described 10. A distinct clinical presentation with plaques and tumors on the back surrounded by erythematous macules and papules expanding centrifugally around the central tumors has been described as ‘reticulohistiocytoma of the dorsum’ or ‘Crosti’s lymphoma’ 11. Association with infections such as Borrelia burgdorferi, HCV, or HHV8 has been described in a few patients 12,13.

Figure 1
Figure 1:
Cutaneous follicle center lymphoma: erythematous infiltrated plaque on the front.


The epidermis is spared as a rule. There is a nodular or a diffuse infiltrate with a predominance of centroblasts and centrocytes admixed with small reactive lymphocytes in the dermis, sometimes also involving the subcutis. According to the pattern of growth, three main types are described: follicular, diffuse, and mixed. In the follicular type, the neoplastic follicles show a reduced mantle zone, lack of tangible body macrophages, and a monomorphous appearance (Fig. 2) 2. A rare variant with a spindle cell morphology has also been described 14.

Figure 2
Figure 2:
Cutaneous follicle center lymphoma with a typical follicular grow pattern (hematoxylin and eosin, ×10).

Immunophenotype, polymerase chain reaction, and molecular genetics

Neoplastic cells are positive for CD20 and CD79. FCL with a follicular pattern of growth are positive for CD21, CD10, and Bcl-6. The presence of positive CD10 and Bcl-6 cells in the interfollicular areas can be observed in most of these cases. FCL with a diffuse pattern of growth are negative for CD10 and do not show a network of CD21 follicular dendritic cells in the background 15,16. Negativity for Bcl-2, MUM-1, and FOX-P3 is usually observed in both the follicular and the diffuse type 17. MIB-1 staining shows reduced proliferation in neoplastic follicles (<50%). Monoclonal rearrangement of the IGH gene is detected in the majority of cases 2, t(14;18) translocation is usually absent 18.

Differential diagnosis

PCFCL with a diffuse pattern of growth should be carefully differentiated by DLBCL-leg as the prognostic features are completely different.


Complete staging investigations are necessary for a correct diagnosis of PCL, excluding a secondary cutaneous involvement from a nodal lymphoma. The TNM classification system has to be applied 19.


Surgical excision, followed by radiotherapy on the field of the excision and on the surrounding skin or radiotherapy alone frequently represents the first choice for most patients 1,20. Systemic anti-CD20 monoclonal antibody (rituximab) is becoming one of the main treatment options for patients with PCFCL, alone or in combination with systemic chemotherapy 21,22. Radioimmunotherapy has also been attempted. Rituximab and interferon-α can also be applied as intralesional agents, but less frequently compared with other treatment modalities 2.


Irrespective of the type of growth, PCFCL has a 5-year survival rate of more than 95%. Bcl-2 expression in PCFCL of the diffuse type has been associated with a poor prognosis 23. Interestingly, PCFCL on the legs has a poor prognosis similar to DLBCL-leg and it is debated whether this is not a phenotypic variant of DLBCL-leg.

Cutaneous marginal zone lymphoma

CMZL is different from extranodal MZL (which is different from nodal MZL and splenic MZL) and has probably been incorrectly classified under the MALT lymphoma in the WHO classification (in fact, the skin is not a MALT organ) 2,4. Complete staging investigations are necessary for the diagnosis of primary cutaneous disease 1,24.

Clinical features

Three variants are identified: conventional, lymphoplasmacytic (formerly cutaneous immunocytoma), and plasmacytic variant (formerly cutaneous plasmacytoma) 2.

Conventional CMZL: Patients are children or younger adults. They present with a solitary or multiple red to reddish-brown papules, plaques, or nodules localized particularly to the upper extremities or the trunk (Fig. 3).

Figure 3
Figure 3:
Cutaneous marginal zone lymphoma: solitary erythematous nodule on the back.

Lymphoplasmacytic CMZL: patients more often are elderly, with single erythematous, reddish-brown, not ulcerated plaques or nodules on the lower extremities. An association with B. burgdorferi is more frequent than in the conventional variant.

Plasmacytic CMZL: The presentation is similar to the conventional variant. This variant is extremely rare and has to be separated by secondary involvement by multiple myeloma.


Conventional MZL: The epidermis is spared. There is a patchy, nodular, or diffuse infiltrate involving the dermis and sometimes the superficial part of the subcutis containing ‘dark’ reactive germinal centers surrounded by a ‘pale-staining’ perifollicular and interfollicular population of small-sized to medium-sized cells with indented nuclei (so-called ‘triphasic’ pattern), inconspicuous nucleoli, and abundant pale cytoplasm [marginal zone (MZ) cells, centrocyte-like cells; Fig. 4]. The neoplastic cells (whose number is variable and can be very low) are MZ cells (usually a proportion, rarely predominant), lymphoplasmacytoid cells, and plasma cells (these are usually arranged at the periphery). In addition, small lymphocytes and occasional large blasts may be observed 2,24. In many cases, reactive B and T lymphocytes, reactive germinal centers, macrophages, and eosinophils represent the majority of the infiltrate 2.

Figure 4
Figure 4:
Cutaneous marginal zone lymphoma: marginal zone cells, lymphoplasmacytoid cells, and some blasts (hematoxylin and eosin, ×400).

Lymphoplasmacytic CMZL: There is a nodular or a diffuse monomorphous infiltrate in the dermis and subcutis. Lymphoplasmacytoid cells and small lymphocytes are predominant. Plasma cells are at the periphery. Eosinophilic intranuclear inclusion (Dutcher bodies) represents a valuable diagnostic indication. In contrast to the conventional variant, here, neoplastic cells are predominant and reactive cells are only a minority. Reactive germinal centers are absent 2.

Plasmacytic CMZL: there are nodules or sheets of cells in the dermis or the subcutis. Mature plasma cells are predominant. Dutcher bodies and Russel bodies are occasionally seen; amyeloid deposit and crystalloid intracytoplasmatic inclusions within macrophages are never found 2.

Immunophenotype and polymerase chain reaction

Conventional MZL: The phenotypic profile of MZ cells is CD20+, CD79a+, Bcl-2+, CD5, CD10, and Bcl-616,25. MIB-1 is positive typically at the periphery of the cellular aggregates. Monoclonal expression of the immunoglobulin light chain is characteristically present. A monoclonal rearrangement of the IGH gene can be observed in approximately 50–60% of cases 2.

Lymphoplasmacytic CMZL: The phenotype is the same as in the conventional type. CD43 may be positive 2.

Plasmacytic CMZL: Most B-cell markers can be negative, but neoplastic cells are positive for CD138 or CD38. Of course, there is a monoclonal expression of one of the immunoglobulin light chains 2.

Differential diagnosis

Rare cases with a predominance of blasts have to be differentiated by PCFCL.


Complete staging investigations are mandatory for a correct diagnosis of PCL, excluding a secondary cutaneous involvement from a nodal lymphoma. The TNM classification system has to be applied 19.


Surgical excision for solitary lesions is the main treatment 20. Radiotherapy is a reliable alternative option. Systemic steroids have been found to be effective in some cases. Patients with multiple lesions may be treated with a systemic infusion of rituximab 22,26. Interferon-α and chlorambucil represent other options for patients with multiple lesions. Interferon-α and rituximab can also be used for intralesional administration 27. Patients with demonstrated infection by B. Burgdorferi can be managed with the administration of systemic antibiotics, with reported cases of a complete response. A ‘watchful waiting’ strategy may be appropriate in many patients. Cases with extracutaneous dissemination should be treated with systemic chemotherapy eventually associated with systemic rituximab 2.


The 5-year survival rate is 98%. Cutaneous recurrences of conventional MZL are frequent 2.

Cutaneous diffuse large B-cell lymphoma, leg type

It is a lymphoma with an intermediate behavior that occurs in approximately 80–85% of cases on the legs in elderly patients 17.

Clinical features

Solitary or clustered erythematous or reddish-brown tumors, frequently ulcerated and mostly located on the distal extremity of one leg, may be observed (Fig. 5). Sometimes, both legs may be involved. Large ulcers can be clinically misdiagnosed as venous leg ulcers. In 10–15% of cases, DLBCL-leg can occur at sites other than the legs 1,28.

Figure 5
Figure 5:
Cutaneous diffuse large B-cell lymphoma, leg type: erythematous nodules and plaques on the leg.


There is a dense, diffuse infiltrate involving the dermis and subcutis. The epidermis is normally spared but some cases may show epidermotropism. Large cells with round nuclei (immunoblasts and centroblasts) represent the neoplastic population (Fig. 6). Mitosis are frequent. Reactive small lymphocytes are usually only sparse 2.

Figure 6
Figure 6:
Cutaneous diffuse large B-cell lymphoma, leg type: dense infiltrate of immunoblasts and centroblasts (hematoxylin and eosin, ×400).

Immunophenotype, molecular genetics, and polymerase chain reaction

DLBCL-leg expresses CD20 and CD79a; bcl-2, MUM-1, and FOX-P1 are positive in the majority of cases. Bcl-6 and CD10 are often expressed, indicating a derivation from germinal center cells 2,29. MIB-1 shows a high proliferation rate. CD30 can be positive in some cases. There is usually a monoclonal population of immunoglobulins. The tumors reveal a monoclonal rearrangement of the IGH gene in the majority of cases. The deletion of 9p21 with inactivation of p16 may have a prognostic significance 30. Microarrays show a different gene-expression profile from nodal DLBCL and from PCFCL diffuse type 31.

Differential diagnosis

PCFCL with a diffuse-type growth is the major differential diagnosis. DLBCL-leg shows large round immunoblasts, whereas FCL of the diffuse type shows large cleaved lymphocytes. FCL of the diffuse type is negative for MUM-1, bcl-2, and FOX-P1, whereas these markers are positive in DLBCL-leg 2.


Complete staging investigations are necessary for a correct diagnosis of PCL, excluding a secondary cutaneous involvement from a nodal lymphoma. The TNM classification system has to be applied 19.


Systemic chemotherapy and rituximab (alone or in combination with chemotherapy) are considered the therapies of choice 20,32. Because of the older age of the patients in most cases, systemic chemotherapy cannot be administered. Local radiotherapy or other less aggressive treatments should be chosen only if one of the previous modalities cannot be applied.


The 5-year survival rate is less than 60%. Local recurrences are frequent and extracutaneous spread may also occur after a few years 2. The deletion of 9p21 with inactivation of p16 may be associated with a more aggressive course 30.

Other cutaneous B-cell lymphomas

Rare types of cutaneous B-cell lymphomas like intravascular large B-cell lymphoma, mantle cell lymphoma, T-cell/histiocyte rich B-cell lymphoma, lymphomatoid granulomatosis, cutaneous EBV+ diffuse large B-cell lymphoma of the elderly and specific cutaneous manifestations of multiple myeloma are not reported in this short review [1,2].

Precursor Hematologic Neoplasm

Blastic plasmacytoid dendritic cell neoplasm

Formerly called ‘blastic NK-cell lymphoma’, CD4+/CD56+ hematodermic cell neoplasm’, ‘early plasmacytoid dendritic cell leukemia-lymphoma,’ and ‘agranular CD4 CD56 hematodermic neoplasm,’ BPDCN is now recognized as the third category of PCL after primary cutaneous B-cell lymphomas and T-cell lymphomas. Neoplastic cells derive probably by the plasmacytoid dendritic cells (also called ‘plasmacytoid monocytes’). BPDCN belong to the group of precursors of hematologic neoplasms because malignant transformation occurs at a very early stage of differentiation 1,2. A relationship with myelogenous leukemia (ML) has been postulated; however, ML has a completely different phenotype and a different gene-expression profile 33.

Clinical features

Interestingly, skin is the only site involved at presentation in many patients, representing an example of the so-called ‘aleukemic leukemia cutis’. Patients, mostly elderly adults, invariably progress to spread of leukemia after variable periods of time 7,34.

Patients present with solitary (rarely) or generalized plaques and tumors with a characteristic ‘bruise-like’ violaceous aspect because of intratumoral hemorrhage. Ulceration is uncommon. The mucosal region may be involved. Only in a proportion of patients (30–40%) are skin lesions accompanied by systemic symptoms and extracutaneous manifestations in the blood, bone marrow, and/or other organs. Thrombocytopenia, anemia, and neutropenia are commonly found. Lymph nodes are involved in about 50% of the cases at presentation 1,2,4,35.


There is a diffuse monomorphous infiltrate of medium-sized neoplastic cells with a blastoid morphology. The epidermis is spared. The involvement of the subcutis is common. In early lesions, there are perivascular infiltrates of blastoid cells, admixed with reactive lymphocytes (Fig. 7) 2. Angiocentricity and/or angiodestruction, necrosis, and granulomatous reactions are uncommon 2.

Figure 7
Figure 7:
Blastic plasmacytoid dendritic cell neoplasm: medium-sized neoplastic cells with blastoid morphology (hematoxylin and eosin, ×400).

Immunophenotype, polymerase chain reaction, and molecular genetics

The neoplastic cells express CD4 and CD56. Terminal deoxynucleotidyl transferase (TdT) is positive in the majority of the cases, whereas myeloid antigens, NK-cell markers, and cytotoxic proteins are negative. CD123 is usually positive, whereas CD3, CD5, CD20, and especially myeloperoxidase are usually negative 7,34. Other positive markers are BDC-A2, CD2AP, ICSBP/IRF8, bcl-2, CD43, CD101, and TCL-1 36. CD4/CD56+ and CD4+/CD56 variants have been described 37.

PCR for TCR and JH are germline. Chromosomal abnormalities have been reported 2,33,38.

Differential diagnosis

ML has to be ruled out with myeloperoxidase staining 2.


Complete staging investigations are necessary. A specific TNM system has been developed 6.


Patients need systemic chemotherapy already in the phase of aleukemic leukemia cutis. The chemotherapy techniques used are those applied for acute leukemias. Remissions after therapy are usually short, with frequent recurrences. Allogenic stem cell transplantation could be a promising treatment, with some cases reported with prolonged survival 39.


The 5-year survival rate is 0% 2.


Conflicts of interest

There are no conflicts of interest.


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blastic plasmacytoid dendritic cell neoplasm; primary cutaneous B-cell lymphomas

© 2012 Egyptian Women's Dermatologic Society