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T Cell Erythroderma (Sezary Syndrome)

R. K. Winkelmann

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Abstract

Acceptance of the Sezary syndrome was slow in the first two decades following its description, and the syndrome was confused with lymphoma cases with erythroderma in many reports. Taswell and Winkelmann1reported seven cases of erythroderma with atypical lymphocytosis in the skin and blood pointing out the clinical, histologic, and cytologic nature of the syndrome. Cases in which lymphoma develops in the course of the Sezary syndrome were later recognized, and in the past decade, cases have been reported from all continents. Winkelmann and Linman2recently summarized the clinical course and response to therapy of 19 patients, noting that this is a definitive form of generalized lymphocytic reticulosis. Two forms of presentation are noted: one following dermatitis and the other an infiltrative erythema. Contact dermatitis delayed hypersensitivity to antigens was demonstrated in three patients who could be tested, and one case of Sezary syndrome appeared after a penicillin-sensitivity

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What this paper is about

Acceptance of the Sezary syndrome was slow in the first two decades following its description, and the syndrome was confused with lymphoma cases with erythroderma in many reports. Taswell and Winkelmann1reported seven cases of erythroderma with atypical lymphocytosis in the skin and blood pointing out the clinical, histologic, and cytologic nature of the syndrome. Cases in which lymphoma develops in the course of the Sezary syndrome were later recognized, and in the past decade, cases have been reported from all continents. Winkelmann and Linman2recently summarized the clinical course and response to therapy of 19 patients, noting that this is a definitive form of generalized lymphocytic reticulosis. Two forms of presentation are noted: one following dermatitis and the other an infiltrative erythema. Contact dermatitis delayed hypersensitivity to antigens was demonstrated in three patients who could be tested, and one case of Sezary syndrome appeared after a penicillin-sensitivity

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Available abstract

Acceptance of the Sezary syndrome was slow in the first two decades following its description, and the syndrome was confused with lymphoma cases with erythroderma in many reports. Taswell and Winkelmann1reported seven cases of erythroderma with atypical lymphocytosis in the skin and blood pointing out the clinical, histologic, and cytologic nature of the syndrome. Cases in which lymphoma develops in the course of the Sezary syndrome were later recognized, and in the past decade, cases have been reported from all continents. Winkelmann and Linman2recently summarized the clinical course and response to therapy of 19 patients, noting that this is a definitive form of generalized lymphocytic reticulosis. Two forms of presentation are noted: one following dermatitis and the other an infiltrative erythema. Contact dermatitis delayed hypersensitivity to antigens was demonstrated in three patients who could be tested, and one case of Sezary syndrome appeared after a penicillin-sensitivity

Key concepts: Erythroderma, Medicine, Dermatology, Exfoliative dermatitis, Mycosis fungoides, Eczematous dermatitis, Lymphoma, Lymphocytosis

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