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Understanding Skin Lymphomas: Clinical Manifestations and Histopathological Features
Overview of Skin Lymphomas
Skin lymphomas are a diverse group of lymphoproliferative diseases characterized by the clonal proliferation of tumor lymphocytes primarily in the skin, with potential secondary spread to other organs such as lymph nodes, blood, spleen, lungs, and liver. Approximately 65% of skin lymphomas originate from mature T-cells, 25% from mature B-cells, and 10% from natural killer (NK) cells.
Mantle Cell Lymphoma Involving Skin
Mantle cell lymphoma (MCL) can present with skin lesions, often as a manifestation of widespread disease. These lesions typically appear as nodules and are characterized by dermal and subcutaneous lymphoid infiltrates. The tumors are composed of small- to medium-sized lymphocytes with irregular nuclear contours, and immunophenotypic studies show positivity for CD20 and cyclin D1. MCL involving the skin is often associated with blastoid cytologic features and may arise primarily in the skin in rare cases.
Diffuse Large Cell Lymphomas of Follicular Center Cell Origin
Diffuse large cell lymphomas of follicular center cell origin can present with nodular and tumorous skin lesions, often confined to a specific area such as the trunk or lower legs. Histologically, these lymphomas show nonepidermotropic diffuse dermal infiltrates consisting mainly of large follicular center cells. Immunophenotypically, they express monotypic surface immunoglobulins and HLA-DR antigens, and are reactive with B-cell-associated monoclonal antisera. This type of lymphoma generally has a favorable prognosis, especially in patients without extracutaneous involvement.
Angiocentric T-cell Lymphoma
Angiocentric T-cell lymphoma is an aggressive form of skin lymphoma distinct from mycosis fungoides. It presents with nodular lesions that may be ulcerated or have intact skin. Histologically, it features a perivascular and peri-adnexal lymphomatous infiltrate, often with invasion of blood vessels and extensive coagulative necrosis. The neoplastic cells exhibit a T-cell phenotype and can vary in size and nuclear morphology. This lymphoma often pursues an aggressive course and may be resistant to treatment.
WHO-EORTC Classification for Primary Cutaneous Lymphomas
The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas includes new entities such as primary cutaneous acral CD8+ T-cell lymphoma and Epstein-Barr virus positive (EBV+) mucocutaneous ulcer. The classification also recognizes different subtypes of primary cutaneous marginal zone lymphomas and modifies the term "primary cutaneous CD4+ small/medium T-cell lymphoma" to "primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder" due to its indolent behavior.
Malignant Lymphoma of the Skin in Children
In children, malignant lymphoma of the skin often presents with solitary nodules, primarily in the head and neck region. The most common histologic types are lymphoblastic lymphoma and large cell lymphoma. These lymphomas can disseminate to other organs, and the prognosis varies depending on the stage at diagnosis. Early-stage patients generally have a better prognosis with prolonged disease-free survival.
Lymphoblastic Lymphoma Presenting in Cutaneous Sites
Lymphoblastic lymphoma can involve cutaneous sites, particularly in the head and neck region. The neoplastic cells are typically small with blastic nuclear chromatin and may exhibit a pre-B cell or T cell immunophenotype. Pre-B cell lymphomas appear to have a predilection for cutaneous involvement.
ALK-positive Anaplastic Large Cell Lymphoma
ALK-positive anaplastic large cell lymphoma (ALCL) limited to the skin is a rare variant in children. These lymphomas express CD30 and ALK proteins and are confined to the skin without systemic involvement. Treatment often involves local measures such as resection or radiotherapy, and the prognosis is generally favorable with complete remission observed in most cases.
Conclusion
Skin lymphomas encompass a wide range of clinical and histopathological presentations. Understanding the specific characteristics and classifications of these lymphomas is crucial for accurate diagnosis and effective treatment. The prognosis varies significantly depending on the type and stage of the lymphoma, highlighting the importance of early detection and appropriate therapeutic strategies.
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