Photopheresis in the Red Man or Pre-Sézary Syndrome

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Abstract
Background: The beneficial effect of extracorporeal photochemotherapy has clearly been established for patients with cutaneous T-cell lymphoma with Sézary’s syndrome. But this treatment has not been used in the red man syndrome or pre-Sézary syndrome. Some of these patients do not respond sufficiently or do not tolerate other therapies. Objective: The purpose of this open study was to evaluate the clinical efficacy of photopheresis in this group of patients. Methods: Seven patients with the red man syndrome were treated on 2 consecutive days every 4 weeks for 6–22 months. Six had been on systemic steroids and 3 had also received retinoids without sufficient effects. All were initially treated with topical nitrogen mustard but no longer tolerated this treatment. Results: All signs of erythroderma disappeared in 6 of the 7 patients. All systemic therapy could be discontinued in all but 2 patients. No side effects were observed. Conclusion: Photopheresis may be an effective alternative treatment for this group of patients, when other types of therapy have failed.

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