Diagnosis, prognosis and management of erythrodermic cutaneous T-cell lymphoma

Erythroderma describes complete or near-complete skin surface erythema of any cause. Cutaneous T-cell lymphoma accounts for a minority of cases of erythroderma and consists of erythrodermic mycosis fungoides, Sézary syndrome. Both adult T-cell leukemia/lymphoma and T-cell prolymphocytic leukemia can also rarely present with erythroderma. Diagnosis may be extremely challenging because benign disorders may have overlapping features with those of lymphoma. Prognosis is poor with median survival of approximately 2 years. The evidence base for therapeutic approaches relies on cohorts and case series and more recently Phase II trials. Improved patient selection and identification of appropriate conditioning regimens for reduced intensity allogeneic hematopoetic transplant are likely to improve survival, although a significant number of patients may not be fit for transplant because of advanced age and comorbidities.