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These studies suggest that skin lymphomas are diverse in type and behavior, ranging from aggressive conditions with potential for widespread organ involvement to those with a more favorable prognosis and potential for remission.
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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.
Angiocentric T-cell lymphoma is a particularly aggressive form of skin lymphoma, distinct from mycosis fungoides. This type of lymphoma is characterized by nodular lesions that may be ulcerated or have intact skin. Histologically, it features a perivascular and peri-adnexal lymphomatous infiltrate predominantly in the mid to deep dermis, often sparing the epidermis and papillary dermis. A notable characteristic is the invasion of blood vessels by lymphoma cells, sometimes accompanied by extensive coagulative necrosis and intraneural invasion.
Diffuse large cell lymphomas of follicular center cell origin often present with nodular and tumorous skin lesions. These lesions are typically confined to a circumscribed area on the trunk or, in elderly women, on the lower legs. Histologically, these lymphomas show nonepidermotropic diffuse dermal infiltrates composed 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.
Mantle cell lymphoma can also involve the skin, often as a manifestation of disseminated disease. Skin lesions in mantle cell lymphoma typically present as dermal and subcutaneous lymphoid infiltrates, with a preference for perivascular and periadnexal areas. These tumors are composed of small- to medium-sized lymphocytes with irregular nuclear contours, and often exhibit blastoid cytologic features. Immunophenotypic studies show positivity for CD20 and cyclin D1, with most cases also positive for CD5.
Skin lymphomas exhibit significant phenotypic heterogeneity. In a study of 56 cases, 49 were T-cell lymphomas and 7 were B-cell lymphomas. Among the T-cell lymphomas, various phenotypes were observed, including helper/inducer, suppressor/cytotoxic, and other mixed phenotypes. This diversity underscores the complexity of skin lymphomas and the importance of precise immunophenotypic characterization for accurate diagnosis and treatment.
Skin lymphomas are a complex and heterogeneous group of diseases with varied clinical presentations and histological features. Understanding the distinct characteristics of different types of skin lymphomas, such as angiocentric T-cell lymphoma, diffuse large cell lymphomas, and mantle cell lymphoma, is crucial for accurate diagnosis and effective treatment. The phenotypic diversity further highlights the need for detailed immunophenotypic analysis in managing these conditions.
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