Primary cutaneous diffuse large B-cell lymphoma, leg type
Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) (also termed PCDLBCL, leg type or primary cutaneous DLBCL, leg type) is a cutaneous lymphoma skin disease that occurs mostly in elderly females. In this disease, B-cells (a type of lymphocyte) become malignant, accumulate in the dermis (i.e. the layer under the epidermis) and subcutaneous tissue below the dermis to form red and violaceous skin nodules and tumors. These lesions typically occur on the lower extremities but in uncommon cases may develop on the skin at virtually any other site. In ~10% of cases, the disease presents with one or more skin lesions none of which are on the lower extremities; the disease in these cases is sometimes regarded as a variant of PCDLBL, LT termed primary cutaneous diffuse large B-cell lymphoma, other (PCDLBC-O). PCDLBCL, LT is a subtype of the diffuse large B-cell lymphomas (DLBCL) and has been thought of as a cutaneous counterpart to them. Like most variants and subtypes of the DLBCL, PCDLBCL, LT is an aggressive malignancy. It has a 5-year overall survival rate of 40-55%, although the PCDLBCL-O variant has a better prognosis than cases in which the legs are involved.

Most lymphomas begin in a lymph node, mucosa-associated lymphoid tissue, the spleen, or another lymphoid tissue within the lymphatic system and then may spread to the skin. In these cases the skin is a secondarily site of involvement. PCDLBC, LT is a primary cutaneous lymphoma, i.e. it begins in the skin and then may spread to lymphoid and/or non-lymphoid tissues in virtually any other site. A suspect PCDLBCL, LT that is not limited to the skin at the time of diagnosis should be regarded as some other variant or subtype of the diffuse large B-cell lymphomas.

PCDLBC, LT represents 5-10% of all primary cutaneous lymphomas. It is to be distinguished from two other primary cutaneous lymphomas that involve B-cells, primary cutaneous follicle center lymphoma (PCMZL) and primary cutaneous marginal zone lymphoma (PCFCL), as well as from a B-cell lymphoma that at diagnosis may appear to be limited to the skin but often is a systemic disease afflicting numerous organs and tissues viz., intravascular large B-cell lymphoma (IVLBCL). These three B-cell lymphomas differ from PCDLBCL, LT in numerous ways but most importantly in their aggressiveness. IVLEBC is, like PCDLBCL, LT, an aggressive disease with a guarded prognoses but unlike the former disease is often widely disseminated at presentation. PCMZL and PCFCL, in contrast, are indolent lymphomas with a relatively good prognoses. Distinguishing between these four cutaneous B-cell lymphomas at the time of diagnosis is critical for their appropriate treatment.
Q1794643
cutaneous lymphoma
UNK
Q949302
skin disease
Concept
Q188930
B-cells
Concept
Q715347
lymphocyte
Concept
Q1483951
malignant
Concept
Q505531
dermis
Concept
Q212743
epidermis
Concept
Q1164665
subcutaneous tissue
Concept
None
diffuse large B-cell lymphomas
UNK
None
overall survival rate
UNK
Q170758
lymph node
Concept
Q288961
mucosa-associated lymphoid tissue
Concept
Q9371
spleen
Concept
Q712604
lymphatic system
Concept
None
variant
UNK
None
subtype
UNK
Q5196683
primary cutaneous lymphomas that involve B-cells
UNK
Q7243121
primary cutaneous marginal zone lymphoma
Concept
Q1414805
systemic disease
Concept
Q6058570
intravascular large B-cell lymphoma
UNK
Q5196683
cutaneous B-cell lymphoma
UNK
Q55780137
Primary cutaneous diffuse large B-cell lymphoma, leg type
Concept
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