Erythrodermic cutaneous T-cell lymphoma: Two case reports / Eritrodermalni kutani limfom T-ćelija - prikaz dva slučaja

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Abstract

Primary cutaneous T-cell lymphomas (CTCLs) are Non-Hodgkin lymphomas where skin may be the only involved organ for a long time. The erythrodermic form of CTCL, including Sezary syndrome, with increased IgE concentration, eosinophilia and intense pruritus, may suggest atopic dermatitis, in the lack of evidence for diagnosis of T-cell lymphoproliferative disorder. After vigorous research, two patients with erythroderma, increased IgE and eosinophilia, were not diagnosed with CTCL. Adult atopic dermatitis was diagnosed, based on clinical examination and histopathologic analysis of the skin sample. Therapy with oral cyclosporin A (CsA) and systemic corticosteroids were initiated, but the improvement was minimal and short-lived. Disease progression was noted in both patients, after a month of cyclosporine therapy: malaise, subfebrile and febrile temperatures, and development of generalized skin nodules were evident. In the first patient (aged 30) repeated examinations confirmed presence of Sezary cells in peripheral blood samples, dominant T-cell clone in the skin, peripheral blood and bone marrow, whereas the last repeated histopathologic analysis revealed T-lymphoproliferative skin disorder. In the second patient, (aged 44) primary cutaneous CD30+ T-cell lymphoma was diagnosed, based on histopathologic analysis of the newly appearing skin nodule. Differential diagnosis of erythroderma is always difficult, since clinical, histopathologic and immunophenotypic findings are frequently insufficient to differentiate between inflammatory and lymphomatous erythroderma. Treatment with cyclosporin A always demands carefull evaluation of the course of the disorder.

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